-
1
-
-
0036843023
-
Secretory pathway quality control operating in Golgi, plasmalemmal, and endosomal systems
-
Arvan P., Zhao X., Ramos-Castaneda J., and Chang A. (2002) Secretory pathway quality control operating in Golgi, plasmalemmal, and endosomal systems. Traffic 3, 771-780.
-
(2002)
Traffic
, vol.3
, pp. 771-780
-
-
Arvan, P.1
Zhao, X.2
Ramos-Castaneda, J.3
Chang, A.4
-
2
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence N. F., Sampat R. M., and Kopito R. R. (2001) Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292, 1552-1555.
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
3
-
-
0033782015
-
Dynamic interaction of BiP, and ER stress transducers in the unfolded-protein response
-
Bertolotti A., Zhang Y., Hendershot L. M., Harding H. P., and Ron D. (2000) Dynamic interaction of BiP, and ER stress transducers in the unfolded-protein response. Nat. Cell Biol. 2, 326-332.
-
(2000)
Nat. Cell Biol.
, vol.2
, pp. 326-332
-
-
Bertolotti, A.1
Zhang, Y.2
Hendershot, L.M.3
Harding, H.P.4
Ron, D.5
-
4
-
-
0027986675
-
Disruption of the compacted myelin sheath of axons of the central nervous system in proteolipid protein-deficient mice
-
Boison D. and Stoffel W. (1994) Disruption of the compacted myelin sheath of axons of the central nervous system in proteolipid protein-deficient mice. Proc. Natl. Acad. Sci. USA 91, 11709-11713.
-
(1994)
Proc. Natl. Acad. Sci. USA
, vol.91
, pp. 11709-11713
-
-
Boison, D.1
Stoffel, W.2
-
6
-
-
0030698129
-
A pathway distinct from the mammalian unfolded protein response regulates expression of endoplasmic reticulum chaperones in non-stressed cells
-
Brewer J. W., Cleveland J. L., and Hendershot L. M. (1997) A pathway distinct from the mammalian unfolded protein response regulates expression of endoplasmic reticulum chaperones in non-stressed cells. EMBO J. 16, 7207-7216.
-
(1997)
EMBO J.
, vol.16
, pp. 7207-7216
-
-
Brewer, J.W.1
Cleveland, J.L.2
Hendershot, L.M.3
-
7
-
-
0033208984
-
ER protein quality control, and proteasome-mediated protein degradation
-
Brodsky J. L. and McCracken A. A. (1999) ER protein quality control, and proteasome-mediated protein degradation. Semin. Cell Dev. Biol. 10, 507-513.
-
(1999)
Semin. Cell Dev. Biol.
, vol.10
, pp. 507-513
-
-
Brodsky, J.L.1
McCracken, A.A.2
-
8
-
-
0030026565
-
Analysis of ATF3, a transcription factor induced by physiological stresses, and modulated by gadd153/Chop10
-
Chen B. P., Wolfgang C. D., and Hai T. (1996) Analysis of ATF3, a transcription factor induced by physiological stresses, and modulated by gadd153/ Chop10. Mol. Cell. Biol. 16, 1157-1168.
-
(1996)
Mol. Cell. Biol.
, vol.16
, pp. 1157-1168
-
-
Chen, B.P.1
Wolfgang, C.D.2
Hai, T.3
-
9
-
-
0029153220
-
Generation, and characterization of a delta F508 cystic fibrosis mouse model
-
Colledge W. H., Abella B. S., Southern K. W., et al. (1995) Generation, and characterization of a delta F508 cystic fibrosis mouse model. Nat. Genet. 10, 445-452.
-
(1995)
Nat. Genet.
, vol.10
, pp. 445-452
-
-
Colledge, W.H.1
Abella, B.S.2
Southern, K.W.3
-
10
-
-
0031972929
-
Overloaded endoplasmic reticulum-Golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22
-
D'Urso D., Prior R., Greiner-Petter R., Gabreels-Festen A. A., and Muller H. W. (1998) Overloaded endoplasmic reticulum-Golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22. J. Neurosci. 18, 731-740.
-
(1998)
J. Neurosci.
, vol.18
, pp. 731-740
-
-
D'Urso, D.1
Prior, R.2
Greiner-Petter, R.3
Gabreels-Festen, A.A.4
Muller, H.W.5
-
11
-
-
0036154050
-
Disease-associated mutations in L1 CAM interfere with ligand interactions, and cell-surface expression
-
De Angelis E., Watkins A., Schafer M., Brummendorf T., and Kenwrick S. (2002) Disease-associated mutations in L1 CAM interfere with ligand interactions, and cell-surface expression. Hum. Mol. Genet. 11, 1-12.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 1-12
-
-
De Angelis, E.1
Watkins, A.2
Schafer, M.3
Brummendorf, T.4
Kenwrick, S.5
-
12
-
-
0036167612
-
Molecular pathways of protein synthesis inhibition during brain reperfusion: Implications for neuronal survival or death
-
DeGracia D. J., Kumar R., Owen C. R., Krause G. S., and White B. C. (2002) Molecular pathways of protein synthesis inhibition during brain reperfusion: implications for neuronal survival or death. J. Cereb. Blood Flow Metab. 22, 127-141.
-
(2002)
J. Cereb. Blood Flow Metab.
, vol.22
, pp. 127-141
-
-
DeGracia, D.J.1
Kumar, R.2
Owen, C.R.3
Krause, G.S.4
White, B.C.5
-
13
-
-
0034425698
-
EIF2AK3, encoding translation initiation factor 2-alpha kinase 3, is mutated in patients with Wolcott-Rallison syndrome
-
Delepine M., Nicolino M., Barrett T., et al. (2000) EIF2AK3, encoding translation initiation factor 2-alpha kinase 3, is mutated in patients with Wolcott-Rallison syndrome. Nat. Genet. 25, 406-409.
-
(2000)
Nat. Genet.
, vol.25
, pp. 406-409
-
-
Delepine, M.1
Nicolino, M.2
Barrett, T.3
-
14
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning G. M., Anderson M. P., Amara J. F., et al. (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358, 761-764.
-
(1992)
Nature
, vol.358
, pp. 761-764
-
-
Denning, G.M.1
Anderson, M.P.2
Amara, J.F.3
-
15
-
-
0036294646
-
Human caspase 12 has acquired deleterious mutations
-
Fischer H., Koenig U., Eckhart L., and Tschachler E. (2002) Human caspase 12 has acquired deleterious mutations. Biochem. Biophys. Res. Commun. 293, 722-726.
-
(2002)
Biochem. Biophys. Res. Commun.
, vol.293
, pp. 722-726
-
-
Fischer, H.1
Koenig, U.2
Eckhart, L.3
Tschachler, E.4
-
16
-
-
76949131802
-
Proteolipides, a new type of tissue lipoproteins. Their isolation from brain
-
Folch J. and Lees M. (1951) Proteolipides, a new type of tissue lipoproteins. Their isolation from brain. J. Biol. Chem. 191, 807-817.
-
(1951)
J. Biol. Chem.
, vol.191
, pp. 807-817
-
-
Folch, J.1
Lees, M.2
-
17
-
-
0028876309
-
CRASH syndrome: Clinical spectrum of corpus callosum hypoplasia, retardation, adducted thumbs, spastic paraparesis, and hydrocephalus due to mutations in one single gene, L1
-
Fransen E., Lemmon V., Van Camp G., et al. (1995) CRASH syndrome: clinical spectrum of corpus callosum hypoplasia, retardation, adducted thumbs, spastic paraparesis, and hydrocephalus due to mutations in one single gene, L1. Eur. J. Hum. Genet. 3, 273-284.
-
(1995)
Eur. J. Hum. Genet.
, vol.3
, pp. 273-284
-
-
Fransen, E.1
Lemmon, V.2
Van Camp, G.3
-
18
-
-
0036189424
-
Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination, and inflammation
-
Garbern J. Y., Yool D. A., Moore G. J., et al. (2002) Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination, and inflammation. Brain 125, 551-561.
-
(2002)
Brain
, vol.125
, pp. 551-561
-
-
Garbern, J.Y.1
Yool, D.A.2
Moore, G.J.3
-
19
-
-
0022168489
-
Using recombinant DNA techniques to study protein targeting in the eukaryotic cell
-
Garoff H. (1985) Using recombinant DNA techniques to study protein targeting in the eukaryotic cell. Annu. Rev. Cell Biol. 1, 403-445.
-
(1985)
Annu. Rev. Cell Biol.
, vol.1
, pp. 403-445
-
-
Garoff, H.1
-
20
-
-
0036896021
-
Rescuing protein conformation: Prospects for pharmacological therapy in cystic fibrosis
-
Gelman M. S. and Kopito R. R. (2002) Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis. J. Clin. Invest. 110, 1591-1597.
-
(2002)
J. Clin. Invest.
, vol.110
, pp. 1591-1597
-
-
Gelman, M.S.1
Kopito, R.R.2
-
21
-
-
0022552149
-
Expression of wild-type, and mutant forms of influenza hemagglutinin: The role of folding in intracellular transport
-
Gething M.-J., McCammon K., and Sambrook J. (1986) Expression of wild-type, and mutant forms of influenza hemagglutinin: the role of folding in intracellular transport. Cell 46, 939-950.
-
(1986)
Cell
, vol.46
, pp. 939-950
-
-
Gething, M.-J.1
McCammon, K.2
Sambrook, J.3
-
22
-
-
0026584271
-
Protein folding in the cell
-
Gething M.-J. and Sambrook J. (1992) Protein folding in the cell. Nature 355, 33-45.
-
(1992)
Nature
, vol.355
, pp. 33-45
-
-
Gething, M.-J.1
Sambrook, J.2
-
23
-
-
0025382820
-
Transport, and assembly processes in the endoplasmic reticulum
-
Gething M.-J. and Sambrook J. (1990) Transport, and assembly processes in the endoplasmic reticulum. Semin. Cell Biol. 1, 65-72.
-
(1990)
Semin. Cell Biol.
, vol.1
, pp. 65-72
-
-
Gething, M.-J.1
Sambrook, J.2
-
24
-
-
0024838247
-
Protein folding, and intracellular transport: Evaluation of conformational changes in nascent exocytotic proteins
-
Gething M.-J., McCammon K., and Sambrook J. (1989) Protein folding, and intracellular transport: evaluation of conformational changes in nascent exocytotic proteins. Meth. Cell Biol. 32, 185-206.
-
(1989)
Meth. Cell Biol.
, vol.32
, pp. 185-206
-
-
Gething, M.-J.1
McCammon, K.2
Sambrook, J.3
-
25
-
-
0032559544
-
Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease
-
Gow A., Southwood C. M., and Lazzarini R. A. (1998) Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J. Cell Biol. 140, 925-934.
-
(1998)
J. Cell Biol.
, vol.140
, pp. 925-934
-
-
Gow, A.1
Southwood, C.M.2
Lazzarini, R.A.3
-
26
-
-
0031025729
-
Conservation of topology, but not conformation, of the proteolipid proteins of the myelin sheath
-
Gow A., Gragerov A., Gard A., Colman D. R., and Lazzarini R. A. (1997) Conservation of topology, but not conformation, of the proteolipid proteins of the myelin sheath. J. Neurosci. 17, 181-189.
-
(1997)
J. Neurosci.
, vol.17
, pp. 181-189
-
-
Gow, A.1
Gragerov, A.2
Gard, A.3
Colman, D.R.4
Lazzarini, R.A.5
-
27
-
-
0030036917
-
A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease
-
Gow A. and Lazzarini R. A. (1996) A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease. Nat. Genet. 13, 422-428.
-
(1996)
Nat. Genet.
, vol.13
, pp. 422-428
-
-
Gow, A.1
Lazzarini, R.A.2
-
28
-
-
0028287054
-
Intracellular transport, and sorting of the oligodendrocyte transmembrane proteolipid protein
-
Gow A., Friedrich V. L., and Lazzarini R. A. (1994a) Intracellular transport, and sorting of the oligodendrocyte transmembrane proteolipid protein. J. Neurosci. Res. 37, 563-573.
-
(1994)
J. Neurosci. Res.
, vol.37
, pp. 563-573
-
-
Gow, A.1
Friedrich, V.L.2
Lazzarini, R.A.3
-
29
-
-
0028226949
-
Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport
-
Gow A., Friedrich V. L., and Lazzarini R. A. (1994b) Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport. J. Neurosci. Res. 37, 574-583.
-
(1994)
J. Neurosci. Res.
, vol.37
, pp. 574-583
-
-
Gow, A.1
Friedrich, V.L.2
Lazzarini, R.A.3
-
30
-
-
0032486428
-
Axonal swellings, and degeneration in mice lacking the major proteolipid of myelin
-
Griffiths I., Klugmann M., Anderson T., et al. (1998) Axonal swellings, and degeneration in mice lacking the major proteolipid of myelin. Science 280, 1610-1613.
-
(1998)
Science
, vol.280
, pp. 1610-1613
-
-
Griffiths, I.1
Klugmann, M.2
Anderson, T.3
-
31
-
-
0036759150
-
Myelin proteolipid protein forms a complex with integrins, and may participate in integrin receptor signaling in oligodendrocytes
-
Gudz T. I., Schneider T. E., Haas T. A., and Macklin W. B. (2002) Myelin proteolipid protein forms a complex with integrins, and may participate in integrin receptor signaling in oligodendrocytes. J. Neurosci. 22, 7398-7407.
-
(2002)
J. Neurosci.
, vol.22
, pp. 7398-7407
-
-
Gudz, T.I.1
Schneider, T.E.2
Haas, T.A.3
Macklin, W.B.4
-
32
-
-
0029094253
-
Quality control in the secretory pathway
-
Hammond C. and Helenius A. (1995) Quality control in the secretory pathway. Curr. Op. Cell Biol. 7, 523-529.
-
(1995)
Curr. Op. Cell Biol.
, vol.7
, pp. 523-529
-
-
Hammond, C.1
Helenius, A.2
-
33
-
-
0036437307
-
Transcriptional, and Translational Control in the Mammalian Unfolded Protein Response
-
Harding H. P., Calfon M., Urano F., Novoa I., and Ron D. (2002) Transcriptional, and Translational Control in the Mammalian Unfolded Protein Response. Annu. Rev. Cell Dev. Biol. 18, 18.
-
(2002)
Annu. Rev. Cell Dev. Biol.
, vol.18
, pp. 18
-
-
Harding, H.P.1
Calfon, M.2
Urano, F.3
Novoa, I.4
Ron, D.5
-
34
-
-
0033634654
-
Regulated translation initiation controls stress-induced gene expression in mammalian cells
-
Harding H. P., Novoa I., Zhang Y., et al. (2000) Regulated translation initiation controls stress-induced gene expression in mammalian cells. Mol. Cell 6, 1099-1108.
-
(2000)
Mol. Cell
, vol.6
, pp. 1099-1108
-
-
Harding, H.P.1
Novoa, I.2
Zhang, Y.3
-
35
-
-
0033862578
-
Insights from mouse models into the molecular basis of neurodegeneration
-
Heintz N. and Zoghbi H. Y. (2000) Insights from mouse models into the molecular basis of neurodegeneration. Annu. Rev. Physiol. 62, 779-802.
-
(2000)
Annu. Rev. Physiol.
, vol.62
, pp. 779-802
-
-
Heintz, N.1
Zoghbi, H.Y.2
-
36
-
-
0034711138
-
Mutations in noncoding regions of the proteolipid protein gene in Pelizaeus-Merzbacher disease
-
Hobson G. M., Davis A. P., Stowell N. C., et al. (2000) Mutations in noncoding regions of the proteolipid protein gene in Pelizaeus-Merzbacher disease. Neurology 55, 1089-1096.
-
(2000)
Neurology
, vol.55
, pp. 1089-1096
-
-
Hobson, G.M.1
Davis, A.P.2
Stowell, N.C.3
-
37
-
-
0001780104
-
Amino acid substitutions in proteolipid protein that cause dysmyelination
-
(Martenson R. E., ed.). CRC Press, Boca Raton
-
Hudson L. D. and Nadon N. L. (1992) Amino acid substitutions in proteolipid protein that cause dysmyelination, in Myelin: Biology, and Chemistry (Martenson R. E., ed.), pp 677-702. CRC Press, Boca Raton.
-
(1992)
Myelin: Biology, and Chemistry
, pp. 677-702
-
-
Hudson, L.D.1
Nadon, N.L.2
-
38
-
-
0031045876
-
Retinopathy induced in mice by targeted disruption of the rhodopsin gene
-
Humphries M. M., Rancourt D., Farrar G. J., et al. (1997) Retinopathy induced in mice by targeted disruption of the rhodopsin gene. Nat. Genet. 15, 216-219.
-
(1997)
Nat. Genet.
, vol.15
, pp. 216-219
-
-
Humphries, M.M.1
Rancourt, D.2
Farrar, G.J.3
-
39
-
-
0024461745
-
Protein oligomerization in the endoplasmic reticulum
-
Hurtley S. M. and Helenius A. (1989) Protein oligomerization in the endoplasmic reticulum. Annu. Rev. Cell Biol. 5, 277-307.
-
(1989)
Annu. Rev. Cell Biol.
, vol.5
, pp. 277-307
-
-
Hurtley, S.M.1
Helenius, A.2
-
40
-
-
0024400060
-
Interactions of misfolded influenza virus hemagglutinin with binding protein (BiP)
-
Hurtley S. M., Bole D. G., Hoover-Litty H., Helenius A., and Copeland C. S. (1989) Interactions of misfolded influenza virus hemagglutinin with binding protein (BiP). J. Cell Biol. 108, 2117-2126.
-
(1989)
J. Cell Biol.
, vol.108
, pp. 2117-2126
-
-
Hurtley, S.M.1
Bole, D.G.2
Hoover-Litty, H.3
Helenius, A.4
Copeland, C.S.5
-
41
-
-
0035967883
-
An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin
-
Imai Y., Soda M., Inoue H., et al. (2001) An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin. Cell 105, 891-902.
-
(2001)
Cell
, vol.105
, pp. 891-902
-
-
Imai, Y.1
Soda, M.2
Inoue, H.3
-
42
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
Jensen T. J., Loo M. A., Pind S., et al. (1995) Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83, 129-135.
-
(1995)
Cell
, vol.83
, pp. 129-135
-
-
Jensen, T.J.1
Loo, M.A.2
Pind, S.3
-
43
-
-
0032576605
-
Aggresomes: A cellular response to misfolded proteins
-
Johnston J. A., Ward C. L., and Kopito R. R. (1998) Aggresomes: a cellular response to misfolded proteins. J. Cell Biol. 143, 1883-1898.
-
(1998)
J. Cell Biol.
, vol.143
, pp. 1883-1898
-
-
Johnston, J.A.1
Ward, C.L.2
Kopito, R.R.3
-
44
-
-
0035504022
-
Inhibition of CHOP translation by a peptide encoded by an open reading frame localized in the chop 5′UTR
-
Jousse C., Bruhat A., Carraro V., et al. (2001) Inhibition of CHOP translation by a peptide encoded by an open reading frame localized in the chop 5′UTR. Nucleic Acids Res. 29, 4341-4351.
-
(2001)
Nucleic Acids Res.
, vol.29
, pp. 4341-4351
-
-
Jousse, C.1
Bruhat, A.2
Carraro, V.3
-
45
-
-
0029845073
-
Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): Evidence that PLP misfolding underlies dysmyelination in mutant mice
-
Jung M., Sommer I., Schachner M., and Nave K. A. (1996) Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): evidence that PLP misfolding underlies dysmyelination in mutant mice. J. Neurosci. 16, 7920-7929.
-
(1996)
J. Neurosci.
, vol.16
, pp. 7920-7929
-
-
Jung, M.1
Sommer, I.2
Schachner, M.3
Nave, K.A.4
-
46
-
-
0028133486
-
Glial cell degeneration, and hypomyelination caused by overexpression of myelin proteolipid protein gene
-
Kagawa T., Ikenaka K., Inoue Y., et al. (1994) Glial cell degeneration, and hypomyelination caused by overexpression of myelin proteolipid protein gene. Neuron 13, 427-442.
-
(1994)
Neuron
, vol.13
, pp. 427-442
-
-
Kagawa, T.1
Ikenaka, K.2
Inoue, Y.3
-
47
-
-
0032588980
-
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis
-
Kalin N., Claass A., Sommer M., Puchelle E., and Tummler B. (1999) DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J. Clin. Invest. 103, 1379-1389.
-
(1999)
J. Clin. Invest.
, vol.103
, pp. 1379-1389
-
-
Kalin, N.1
Claass, A.2
Sommer, M.3
Puchelle, E.4
Tummler, B.5
-
48
-
-
0030936662
-
Myelin proteolipid protein expressed in COS-1 cells is targeted to actin-associated surfaces
-
Kalwy S. A., Smith R., and Kidd G. J. (1997) Myelin proteolipid protein expressed in COS-1 cells is targeted to actin-associated surfaces. J. Neurosci. Res. 48, 201-211.
-
(1997)
J. Neurosci. Res.
, vol.48
, pp. 201-211
-
-
Kalwy, S.A.1
Smith, R.2
Kidd, G.J.3
-
49
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
Kartner N., Augustinas O., Jensen T. J., Naismith A. L., and Riordan J. R. (1992) Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat. Genet. 1, 321-327.
-
(1992)
Nat. Genet.
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
50
-
-
0033258544
-
Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response
-
Katayama T., Imaizumi K., Sato N., et al. (1999) Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response. Nat. Cell Biol. 1, 479-485.
-
(1999)
Nat. Cell Biol.
, vol.1
, pp. 479-485
-
-
Katayama, T.1
Imaizumi, K.2
Sato, N.3
-
51
-
-
0033562966
-
Stress signaling from the lumen of the endoplasmic reticulum: Coordination of gene transcriptional, and translational controls
-
Kaufman R. J. (1999) Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional, and translational controls. Genes Dev. 13, 1211-1233.
-
(1999)
Genes Dev.
, vol.13
, pp. 1211-1233
-
-
Kaufman, R.J.1
-
52
-
-
0036853914
-
Orchestrating the unfolded protein response in health, and disease
-
Kaufman R. J. (2002) Orchestrating the unfolded protein response in health, and disease. J. Clin. Invest. 110, 1389-1398.
-
(2002)
J. Clin. Invest.
, vol.110
, pp. 1389-1398
-
-
Kaufman, R.J.1
-
53
-
-
0033005366
-
Eukaryotic initiation factor eIF2
-
Kimball S. R. (1999) Eukaryotic initiation factor eIF2. Int. J. Biochem. Cell Biol. 31, 25-29.
-
(1999)
Int. J. Biochem. Cell Biol.
, vol.31
, pp. 25-29
-
-
Kimball, S.R.1
-
54
-
-
0025041029
-
Protein degradation in the endoplasmic reticulum
-
Klausner R. D. and Sitia R. (1990) Protein degradation in the endoplasmic reticulum. Cell 62, 611-614.
-
(1990)
Cell
, vol.62
, pp. 611-614
-
-
Klausner, R.D.1
Sitia, R.2
-
55
-
-
0031037761
-
Assembly of CNS myelin in the absence of proteolipid protein
-
Klugmann M., Schwab M. H., Puhlhofer A., et a. (1997) Assembly of CNS myelin in the absence of proteolipid protein. Neuron 18, 59-70.
-
(1997)
Neuron
, vol.18
, pp. 59-70
-
-
Klugmann, M.1
Schwab, M.H.2
Puhlhofer, A.3
-
56
-
-
0028236505
-
The rumpshaker mutation in spastic paraplegia
-
Kobayashi H., Hoffman E. P., and Marks H. G. (1994) The rumpshaker mutation in spastic paraplegia. Nat. Genet. 7, 351-352.
-
(1994)
Nat. Genet.
, vol.7
, pp. 351-352
-
-
Kobayashi, H.1
Hoffman, E.P.2
Marks, H.G.3
-
57
-
-
0034278759
-
Aggresomes, and Russell bodies. Symptoms of cellular indigestion?
-
Kopito R. R. and Sitia R. (2000) Aggresomes, and Russell bodies. Symptoms of cellular indigestion? EMBO Rep. 1, 225-231.
-
(2000)
EMBO Rep.
, vol.1
, pp. 225-231
-
-
Kopito, R.R.1
Sitia, R.2
-
58
-
-
0023852783
-
The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins
-
Kozutsumi Y., Segal M., Normington K., Gething M. J., and Sambrook J. (1988) The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins. Nature 332, 462-464.
-
(1988)
Nature
, vol.332
, pp. 462-464
-
-
Kozutsumi, Y.1
Segal, M.2
Normington, K.3
Gething, M.J.4
Sambrook, J.5
-
59
-
-
0034996080
-
Brain ischemia, and reperfusion activates the eukaryotic initiation factor 2alpha kinase, PERK
-
Kumar R., Azam S., Sullivan J. M., et al. (2001) Brain ischemia, and reperfusion activates the eukaryotic initiation factor 2alpha kinase, PERK. J. Neurochem. 77, 1418-1421.
-
(2001)
J. Neurochem.
, vol.77
, pp. 1418-1421
-
-
Kumar, R.1
Azam, S.2
Sullivan, J.M.3
-
60
-
-
0033208952
-
Procollagen folding, and assembly: The role of endoplasmic reticulum enzymes, and molecular chaperones
-
Lamande S. R. and Bateman J. F. (1999) Procollagen folding, and assembly: the role of endoplasmic reticulum enzymes, and molecular chaperones. Semin. Cell Dev. Biol. 10, 455-464.
-
(1999)
Semin. Cell Dev. Biol.
, vol.10
, pp. 455-464
-
-
Lamande, S.R.1
Bateman, J.F.2
-
61
-
-
0028341304
-
Association between calnexin, and a secretion-incompetent variant of human α-antitrypsin
-
Le A., Steiners J. L., Ferrell G. A., Shaker J. C., and Sifers R. N. (1994) Association between calnexin, and a secretion-incompetent variant of human α-antitrypsin. J. Biol. Chem. 269, 7514-7519.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 7514-7519
-
-
Le, A.1
Steiners, J.L.2
Ferrell, G.A.3
Shaker, J.C.4
Sifers, R.N.5
-
62
-
-
0035423875
-
The glucose-regulated proteins: Stress induction, and clinical applications
-
Lee A. S. (2001) The glucose-regulated proteins: stress induction, and clinical applications. Trends. Biochem. Sci. 26, 504-510.
-
(2001)
Trends. Biochem. Sci.
, vol.26
, pp. 504-510
-
-
Lee, A.S.1
-
63
-
-
0037083755
-
IRE1-mediated unconventional mRNA splicing, and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response
-
Lee K., Tirasophon W., Shen X., et al. (2002) IRE1-mediated unconventional mRNA splicing, and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response. Genes Dev. 16, 452-466.
-
(2002)
Genes Dev.
, vol.16
, pp. 452-466
-
-
Lee, K.1
Tirasophon, W.2
Shen, X.3
-
64
-
-
18344386777
-
Subunits of the translation initiation factor eIF2B are mutant in leukoencephalopathy with vanishing white matter
-
Leegwater P. A., Vermeulen G., Konst A. A., et al. (2001) Subunits of the translation initiation factor eIF2B are mutant in leukoencephalopathy with vanishing white matter. Nat. Genet. 29, 383-388.
-
(2001)
Nat. Genet.
, vol.29
, pp. 383-388
-
-
Leegwater, P.A.1
Vermeulen, G.2
Konst, A.A.3
-
65
-
-
0029012309
-
Alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization
-
Lomas D. A., Elliott P. R., Sidhar S. K., et al. (1995) alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization. J. Biol. Chem. 270, 16864-16870.
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 16864-16870
-
-
Lomas, D.A.1
Elliott, P.R.2
Sidhar, S.K.3
-
66
-
-
0037195617
-
Conversion of PrP to a Self-Perpetuating PrPSc-like Conformation in the Cytosol
-
Ma J. and Lindquist S. (2002) Conversion of PrP to a Self-Perpetuating PrPSc-like Conformation in the Cytosol. Science 298, 1785-1788.
-
(2002)
Science
, vol.298
, pp. 1785-1788
-
-
Ma, J.1
Lindquist, S.2
-
67
-
-
0037195647
-
Neurotoxicity, and Neurodegeneration When PrP Accumulates in the Cytosol
-
Ma J., Wollmann R., and Lindquist S. (2002a) Neurotoxicity, and Neurodegeneration When PrP Accumulates in the Cytosol. Science 298, 1781-1785.
-
(2002)
Science
, vol.298
, pp. 1781-1785
-
-
Ma, J.1
Wollmann, R.2
Lindquist, S.3
-
68
-
-
0035966320
-
The unfolding tale of the unfolded protein response
-
Ma Y. and Hendershot L. M. (2001) The unfolding tale of the unfolded protein response. Cell 107, 827-830.
-
(2001)
Cell
, vol.107
, pp. 827-830
-
-
Ma, Y.1
Hendershot, L.M.2
-
69
-
-
0036314984
-
Two distinct stress signaling pathways converge upon the CHOP promoter during the mammalian unfolded protein response
-
Ma Y., Brewer J. W., Diehl J. A., and Hendershot L. M. (2002b) Two distinct stress signaling pathways converge upon the CHOP promoter during the mammalian unfolded protein response. J. Mol. Biol. 318, 1351-1365.
-
(2002)
J. Mol. Biol.
, vol.318
, pp. 1351-1365
-
-
Ma, Y.1
Brewer, J.W.2
Diehl, J.A.3
Hendershot, L.M.4
-
70
-
-
0042812063
-
Frame switch splicing, and regulated intramembrane proteolysis: Key words to understand the unfolded protein response
-
Mori K. (2003) Frame switch splicing, and regulated intramembrane proteolysis: key words to understand the unfolded protein response. Traffic 4, 519-528.
-
(2003)
Traffic
, vol.4
, pp. 519-528
-
-
Mori, K.1
-
71
-
-
0034255327
-
Clinical mutations in the L1 neural cell adhesion molecule affect cell-surface expression
-
Moulding H. D., Martuza R. L., and Rabkin S. D. (2000) Clinical mutations in the L1 neural cell adhesion molecule affect cell-surface expression. J. Neurosci. 20, 5696-5702.
-
(2000)
J. Neurosci.
, vol.20
, pp. 5696-5702
-
-
Moulding, H.D.1
Martuza, R.L.2
Rabkin, S.D.3
-
72
-
-
0030900850
-
Aberrant protein trafficking in Trembler suggests a disease mechanism for hereditary human peripheral neuropathies
-
Naef R., Adlkofer K., Lescher B., and Suter U. (1997) Aberrant protein trafficking in Trembler suggests a disease mechanism for hereditary human peripheral neuropathies. Mol. Cell. Neurosci. 9, 13-25.
-
(1997)
Mol. Cell. Neurosci.
, vol.9
, pp. 13-25
-
-
Naef, R.1
Adlkofer, K.2
Lescher, B.3
Suter, U.4
-
73
-
-
0023389399
-
Splice site selectionin the proteolipid protein (PLP) gene transcript, and primary structure of the DM-20 protein of central nervous system myelin
-
Nave K.-A., Lai C., Bloom F. E., and Milner R. J. (1987) Splice site selectionin the proteolipid protein (PLP) gene transcript, and primary structure of the DM-20 protein of central nervous system myelin. Proc. Natl. Acad. Sci. USA 84, 5665-5669.
-
(1987)
Proc. Natl. Acad. Sci. USA
, vol.84
, pp. 5665-5669
-
-
Nave, K.-A.1
Lai, C.2
Bloom, F.E.3
Milner, R.J.4
-
74
-
-
0032158987
-
ASK1 is essential for JNK/SAPK activation by TRAF2
-
Nishitoh H., Saitoh M., Mochida Y., et al. (1998) ASK1 is essential for JNK/SAPK activation by TRAF2. Mol. Cell. 2, 389-395.
-
(1998)
Mol. Cell
, vol.2
, pp. 389-395
-
-
Nishitoh, H.1
Saitoh, M.2
Mochida, Y.3
-
75
-
-
0036606540
-
ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats
-
Nishitoh H., Matsuzawa A., Tobiume K., et al. (2002) ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats. Genes Dev. 16, 1345-1355.
-
(2002)
Genes Dev.
, vol.16
, pp. 1345-1355
-
-
Nishitoh, H.1
Matsuzawa, A.2
Tobiume, K.3
-
76
-
-
0033598996
-
A role for presenilin-1 in nuclear accumulation of Irel fragments, and induction of the mammalian unfolded protein response
-
Niwa M., Sidrauski C., Kaufman R. J., and Walter P. (1999) A role for presenilin-1 in nuclear accumulation of Irel fragments, and induction of the mammalian unfolded protein response. Cell 99, 691-702.
-
(1999)
Cell
, vol.99
, pp. 691-702
-
-
Niwa, M.1
Sidrauski, C.2
Kaufman, R.J.3
Walter, P.4
-
77
-
-
0035947778
-
Feedback inhibition of the unfolded protein response by GADD34-mediated dephosphorylation of eIF2alpha
-
Novoa I., Zeng H., Harding H. P., and Ron D. (2001) Feedback inhibition of the unfolded protein response by GADD34-mediated dephosphorylation of eIF2alpha. J. Cell Biol. 153, 1011-1022.
-
(2001)
J. Cell Biol.
, vol.153
, pp. 1011-1022
-
-
Novoa, I.1
Zeng, H.2
Harding, H.P.3
Ron, D.4
-
78
-
-
0021088280
-
Immunocytochemical demonstration of the transport of myelin proteins through the Golgi apparatus
-
Nussbaum J. L. and Roussel G. (1983) Immunocytochemical demonstration of the transport of myelin proteins through the Golgi apparatus. Cell Tissue Res. 234, 547-559.
-
(1983)
Cell Tissue Res.
, vol.234
, pp. 547-559
-
-
Nussbaum, J.L.1
Roussel, G.2
-
79
-
-
0036713724
-
Distinct roles of ATF6, and PERK in transcription during the mammalian unfolded protein response
-
Okada T., Yoshida H., Akazawa R., Negishi M., and Mori K. (2002) Distinct roles of ATF6, and PERK in transcription during the mammalian unfolded protein response. Biochem. J. 16, 585-594.
-
(2002)
Biochem. J.
, vol.16
, pp. 585-594
-
-
Okada, T.1
Yoshida, H.2
Akazawa, R.3
Negishi, M.4
Mori, K.5
-
80
-
-
0026463972
-
Transgenic mice with a rhodopsin mutation (pro23his): A mouse model of autosomal dominant retinitis pigmentosa
-
Olsson J. E., Gordon J. W., Pawlyk B. S., et al. (1992) Transgenic mice with a rhodopsin mutation (pro23his): a mouse model of autosomal dominant retinitis pigmentosa. Neuron 9, 815-830.
-
(1992)
Neuron
, vol.9
, pp. 815-830
-
-
Olsson, J.E.1
Gordon, J.W.2
Pawlyk, B.S.3
-
81
-
-
0035190132
-
Endoplasmic reticulum dysfunction-a common denominator for cell injury in acute, and degenerative diseases of the brain?
-
Paschen W. and FrandsenA. (2001) Endoplasmic reticulum dysfunction-a common denominator for cell injury in acute, and degenerative diseases of the brain? J. Neurochem. 79, 719-725.
-
(2001)
J. Neurochem.
, vol.79
, pp. 719-725
-
-
Paschen, W.1
Frandsen, A.2
-
82
-
-
0032544514
-
Activation of gadd153 expression through transient cerebral ischemia: Evidence that ischemia causes endoplasmic reticulum dysfunction
-
Paschen W., Gissel C., Linden T., Althausen S., and Doutheil J. (1998) Activation of gadd153 expression through transient cerebral ischemia: evidence that ischemia causes endoplasmic reticulum dysfunction. Brain Res. Mol. Brain Res. 60, 115-122.
-
(1998)
Brain Res. Mol. Brain Res.
, vol.60
, pp. 115-122
-
-
Paschen, W.1
Gissel, C.2
Linden, T.3
Althausen, S.4
Doutheil, J.5
-
83
-
-
0035370949
-
Intracellular signaling from the endoplasmic reticulum to the nucleus: The unfolded protein response in yeast, and mammals
-
Patil C. and Walter P. (2001) Intracellular signaling from the endoplasmic reticulum to the nucleus: the unfolded protein response in yeast, and mammals. Curr. Opin. Cell Biol. 13, 349-355.
-
(2001)
Curr. Opin. Cell Biol.
, vol.13
, pp. 349-355
-
-
Patil, C.1
Walter, P.2
-
84
-
-
0030904230
-
Jimpy-4J mouse has a missense mutation in exon 2 of the Plp gene
-
Pearsall G. B., Nadon N. L., Wolf M. K., and Billings-Gagliardi S. (1997) Jimpy-4J mouse has a missense mutation in exon 2 of the Plp gene. Dev. Neurosci. 19, 337-341.
-
(1997)
Dev. Neurosci.
, vol.19
, pp. 337-341
-
-
Pearsall, G.B.1
Nadon, N.L.2
Wolf, M.K.3
Billings-Gagliardi, S.4
-
85
-
-
0035961291
-
Pathogenesis, diagnosis, and treatment of systemic amyloidosis
-
discussion 210-201
-
Pepys M. B. (2001) Pathogenesis, diagnosis, and treatment of systemic amyloidosis. Philos. Trans. R. Soc. Lond. B. Biol. Sci. 356, 203-210; discussion 210-201.
-
(2001)
Philos. Trans. R. Soc. Lond. B. Biol. Sci.
, vol.356
, pp. 203-210
-
-
Pepys, M.B.1
-
86
-
-
0027193569
-
The oligodendrocyte, and its many cellular processes
-
Pfeiffer S. E., Warrington A. E., and Bansal R. (1993) The oligodendrocyte, and its many cellular processes. Trends Cell Biol. 3, 191-197.
-
(1993)
Trends Cell Biol.
, vol.3
, pp. 191-197
-
-
Pfeiffer, S.E.1
Warrington, A.E.2
Bansal, R.3
-
87
-
-
0029961665
-
Major histocompatibility class I expression in oligodendrocytes induces hypomyelination in transgenic mice
-
Power C., Kong P., and Trapp B. (1996) Major histocompatibility class I expression in oligodendrocytes induces hypomyelination in transgenic mice. J. Neurosci. Res. 44, 165-173.
-
(1996)
J. Neurosci. Res.
, vol.44
, pp. 165-173
-
-
Power, C.1
Kong, P.2
Trapp, B.3
-
89
-
-
0028325902
-
Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage
-
Readhead C., Schneider A., Griffiths I. R., and Nave K.-A. (1994) Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage. Neuron 12, 583-595.
-
(1994)
Neuron
, vol.12
, pp. 583-595
-
-
Readhead, C.1
Schneider, A.2
Griffiths, I.R.3
Nave, K.-A.4
-
90
-
-
0024149621
-
Regulation of protein export from the endoplasmic reticulum
-
Rose J. K. and Doms R. W. (1988) Regulation of protein export from the endoplasmic reticulum. Annu. Rev. Cell Biol. 4, 257-288.
-
(1988)
Annu. Rev. Cell Biol.
, vol.4
, pp. 257-288
-
-
Rose, J.K.1
Doms, R.W.2
-
91
-
-
0023213593
-
Arrest of proteolipid transport through the Golgi apparatus in Jimpy brain
-
Roussel G., Meskovic N. M., Trifilieff E., Artault J.-C., and Nussbaum J.-L. (1987) Arrest of proteolipid transport through the Golgi apparatus in Jimpy brain. J. Neurocytol. 16, 195-204.
-
(1987)
J. Neurocytol.
, vol.16
, pp. 195-204
-
-
Roussel, G.1
Meskovic, N.M.2
Trifilieff, E.3
Artault, J.-C.4
Nussbaum, J.-L.5
-
92
-
-
0037220860
-
The C264Y missense mutation in the extracellular domain of L1 impairs protein trafficking in vitro, and in vivo
-
Runker A. E., Bartsch U., Nave K. A., and Schachner M. (2003) The C264Y missense mutation in the extracellular domain of L1 impairs protein trafficking in vitro, and in vivo. J. Neurosci. 23,277-286.
-
(2003)
J. Neurosci.
, vol.23
, pp. 277-286
-
-
Runker, A.E.1
Bartsch, U.2
Nave, K.A.3
Schachner, M.4
-
93
-
-
0037114971
-
Endoplasmic reticulum stress, and the unfolded protein response in cellular models of Parkinson's disease
-
Ryu E. J., Harding H. P., Angelastro J. M., et al. (2002) Endoplasmic reticulum stress, and the unfolded protein response in cellular models of Parkinson's disease. J. Neurosci. 22, 10690-10698.
-
(2002)
J. Neurosci.
, vol.22
, pp. 10690-10698
-
-
Ryu, E.J.1
Harding, H.P.2
Angelastro, J.M.3
-
94
-
-
0035859867
-
Mutations of peripheral myelin protein 22 result in defective trafficking through mechanisms which may be common to diseases involving tetraspan membrane proteins
-
Sanders C. R., Ismail-Beigi F., and McEnery M. W. (2001) Mutations of peripheral myelin protein 22 result in defective trafficking through mechanisms which may be common to diseases involving tetraspan membrane proteins. Biochemistry 40, 9453-9459.
-
(2001)
Biochemistry
, vol.40
, pp. 9453-9459
-
-
Sanders, C.R.1
Ismail-Beigi, F.2
McEnery, M.W.3
-
95
-
-
0033671650
-
Upregulation of BiP, and CHOP by the unfolded-protein response is independent of presenilin expression
-
Sato N., Urano F., Yoon Leem J., et al. (2000) Upregulation of BiP, and CHOP by the unfolded-protein response is independent of presenilin expression. Nat. Cell Biol. 2, 863-870.
-
(2000)
Nat. Cell Biol.
, vol.2
, pp. 863-870
-
-
Sato, N.1
Urano, F.2
Yoon Leem, J.3
-
96
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
Sato S., Ward C. L., Krouse M. E., Wine J. J., and Kopito R. R. (1996) Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J. Biol. Chem. 271, 635-638.
-
(1996)
J. Biol. Chem.
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
97
-
-
0029079396
-
Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein
-
Schneider A., Griffiths I., Readhead C., and Nave K.-A. (1995) Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein. Proc. Natl. Acad. Sci. USA 92, 4447-4451.
-
(1995)
Proc. Natl. Acad. Sci. USA
, vol.92
, pp. 4447-4451
-
-
Schneider, A.1
Griffiths, I.2
Readhead, C.3
Nave, K.-A.4
-
98
-
-
0026767888
-
Uncoupling of hypomyelination, and glial cell death by a mutation in the proteolipid protein gene
-
Schneider A., Montague P., Griffiths I., et al. (1992) Uncoupling of hypomyelination, and glial cell death by a mutation in the proteolipid protein gene. Nature 358, 758-761.
-
(1992)
Nature
, vol.358
, pp. 758-761
-
-
Schneider, A.1
Montague, P.2
Griffiths, I.3
-
99
-
-
0036069980
-
ER stress regulation of ATF6 localization by dissociation of BiP/GRP78 binding, and unmasking of Golgi localization signals
-
Shen J., Chen X., Hendershot L., and Prywes R. (2002) ER stress regulation of ATF6 localization by dissociation of BiP/GRP78 binding, and unmasking of Golgi localization signals. Dev. Cell. 3, 99-111.
-
(2002)
Dev. Cell
, vol.3
, pp. 99-111
-
-
Shen, J.1
Chen, X.2
Hendershot, L.3
Prywes, R.4
-
100
-
-
0037369640
-
Schwann cell expression of PLP1 but not its alternatively spliced isoform DM20 is necessary to prevent demyelinating peripheral neuropathy
-
Shy M., Hobson G., Boespflug-Tanguy O., et al. (2003) Schwann cell expression of PLP1 but not its alternatively spliced isoform DM20 is necessary to prevent demyelinating peripheral neuropathy. Ann. Neurol., 53, 354-365.
-
(2003)
Ann. Neurol..
, vol.53
, pp. 354-365
-
-
Shy, M.1
Hobson, G.2
Boespflug-Tanguy, O.3
-
101
-
-
0028316886
-
Proteolipid protein interactions in transfectants: Implications for myelin assembly
-
Sinoway M. P., Kitagawa K., Timsit S., Hashim G. A., and Colman D. R. (1994) Proteolipid protein interactions in transfectants: implications for myelin assembly. J. Neurosci. Res. 37, 551-562.
-
(1994)
J. Neurosci. Res.
, vol.37
, pp. 551-562
-
-
Sinoway, M.P.1
Kitagawa, K.2
Timsit, S.3
Hashim, G.A.4
Colman, D.R.5
-
102
-
-
0035869423
-
Molecular mechanisms of disease stemming from mutations in the proteolipid protein gene
-
Southwood C. M. and Gow A. (2001) Molecular mechanisms of disease stemming from mutations in the proteolipid protein gene. Micros. Res. Tech. 52, 700-708.
-
(2001)
Micros. Res. Tech.
, vol.52
, pp. 700-708
-
-
Southwood, C.M.1
Gow, A.2
-
103
-
-
0037079142
-
The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease
-
Southwood C. M., Garbern J., Jiang W., and Gow A. (2002) The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease. Neuron 36, 585-596.
-
(2002)
Neuron
, vol.36
, pp. 585-596
-
-
Southwood, C.M.1
Garbern, J.2
Jiang, W.3
Gow, A.4
-
104
-
-
0034212602
-
The evolution of lipophilin genes from invertebrates to tetrapods: DM-20 cannot replace PLP in CNS myelin
-
Stecca B., Southwood C. M., Gragerov A., et al. (2000) The evolution of lipophilin genes from invertebrates to tetrapods: DM-20 cannot replace PLP in CNS myelin. J. Neurosci. 20, 4002-4010.
-
(2000)
J. Neurosci.
, vol.20
, pp. 4002-4010
-
-
Stecca, B.1
Southwood, C.M.2
Gragerov, A.3
-
105
-
-
0031214489
-
Phenotypic severity of murine Plp mutants reflects in vivo, and in vitro variations in transport of PLP isoproteins
-
Thomson C. E., Montague P., Jung M., Nave K.-A., and Griffiths I. R. (1997) Phenotypic severity of murine Plp mutants reflects in vivo, and in vitro variations in transport of PLP isoproteins. Glia 20, 322-332.
-
(1997)
Glia
, vol.20
, pp. 322-332
-
-
Thomson, C.E.1
Montague, P.2
Jung, M.3
Nave, K.-A.4
Griffiths, I.R.5
-
106
-
-
0033559844
-
Transport of Trembler-J mutant peripheral myelin protein 22 is blocked in the intermediate compartment, and affects the transport of the wild-type protein by direct interaction
-
Tobler A. R., Notterpek L., Naef R., et al. (1999) Transport of Trembler-J mutant peripheral myelin protein 22 is blocked in the intermediate compartment, and affects the transport of the wild-type protein by direct interaction. J. Neurosci. 19, 2027-2036.
-
(1999)
J. Neurosci.
, vol.19
, pp. 2027-2036
-
-
Tobler, A.R.1
Notterpek, L.2
Naef, R.3
-
107
-
-
0025944623
-
Dysmyelination in transgenic mice resulting from expression of class I histocompatibility molecules in oligodendrocytes
-
Turnley A. M., Morahan G., Okano H., et al. (1991) Dysmyelination in transgenic mice resulting from expression of class I histocompatibility molecules in oligodendrocytes. Nature 353, 566-569.
-
(1991)
Nature
, vol.353
, pp. 566-569
-
-
Turnley, A.M.1
Morahan, G.2
Okano, H.3
-
108
-
-
0029928173
-
Stress-induced binding of transcription factor CHOP to a novel DNA- control element
-
Ubeda M., Wang X.-Z., Zinszner H., et al. (1996) Stress-induced binding of transcription factor CHOP to a novel DNA- control element. Mol. Cell. Biol. 16, 1479-1489.
-
(1996)
Mol. Cell. Biol.
, vol.16
, pp. 1479-1489
-
-
Ubeda, M.1
Wang, X.-Z.2
Zinszner, H.3
-
109
-
-
0036403732
-
Prions as protein-based genetic elements
-
Uptain S. M. and Lindquist S. (2002) Prions as protein-based genetic elements. Annu. Rev. Microbiol. 56, 703-741.
-
(2002)
Annu. Rev. Microbiol.
, vol.56
, pp. 703-741
-
-
Uptain, S.M.1
Lindquist, S.2
-
110
-
-
0034723235
-
Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1
-
Urano F., Wang X., Bertolotti A., et al. (2000) Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1. Science 287, 664-666.
-
(2000)
Science
, vol.287
, pp. 664-666
-
-
Urano, F.1
Wang, X.2
Bertolotti, A.3
-
111
-
-
0030975392
-
A new leukoencephalopathy with vanishing white matter
-
van der Knaap M. S., Barth P. G., Gabreels F. J., et al. (1997) A new leukoencephalopathy with vanishing white matter. Neurology 48, 845-855.
-
(1997)
Neurology
, vol.48
, pp. 845-855
-
-
Van der Knaap, M.S.1
Barth, P.G.2
Gabreels, F.J.3
-
112
-
-
0036156978
-
Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter
-
van der Knaap M. S., Leegwater P. A., Konst A. A., et al. (2002) Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter. Ann. Neurol. 51, 264-270.
-
(2002)
Ann. Neurol.
, vol.51
, pp. 264-270
-
-
Van der Knaap, M.S.1
Leegwater, P.A.2
Konst, A.A.3
-
113
-
-
0345367980
-
Understanding glial abnormalities associated with myelin deficiency in the jimpy mutant mouse
-
Vela J. M., Gonzalez B., and Castellano B. (1998) Understanding glial abnormalities associated with myelin deficiency in the jimpy mutant mouse. Brain Res. Brain Res. Rev. 26, 29-42.
-
(1998)
Brain Res. Brain Res. Rev.
, vol.26
, pp. 29-42
-
-
Vela, J.M.1
Gonzalez, B.2
Castellano, B.3
-
114
-
-
8944259440
-
Signals from the stressed endoplasmic reticulum induce C/EBP-homologous protein (CHOP/GADD153)
-
Wang X.-Z., Lawson B., Brewer J. W., et al. (1996) Signals from the stressed endoplasmic reticulum induce C/EBP-homologous protein (CHOP/GADD153). Mol. Cell. Biol. 16, 4273-4280.
-
(1996)
Mol. Cell. Biol.
, vol.16
, pp. 4273-4280
-
-
Wang, X.-Z.1
Lawson, B.2
Brewer, J.W.3
-
115
-
-
0029938805
-
Stress-induced phosphorylation, and activation of the transcription factor CHOP (GADD153) by p38 MAP Kinase
-
Wang X.-Z. and Ron D. (1996) Stress-induced phosphorylation, and activation of the transcription factor CHOP (GADD153) by p38 MAP Kinase. Science 272, 1347-1349.
-
(1996)
Science
, vol.272
, pp. 1347-1349
-
-
Wang, X.-Z.1
Ron, D.2
-
116
-
-
0032127493
-
Identification of novel stress-induced genes downstream of chop
-
Wang X.-Z., Kuroda M., Sok J., et al. (1998) Identification of novel stress-induced genes downstream of chop. EMBO J. 17, 3619-3630.
-
(1998)
EMBO J.
, vol.17
, pp. 3619-3630
-
-
Wang, X.-Z.1
Kuroda, M.2
Sok, J.3
-
117
-
-
0026980191
-
Proteolipid protein (PLP) of CNS myelin: Positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues, and implications for the membrane topology of PLP
-
Weimbs T. and Stoffel W. (1992) Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues, and implications for the membrane topology of PLP. Biochemistry 31, 12289-12296.
-
(1992)
Biochemistry
, vol.31
, pp. 12289-12296
-
-
Weimbs, T.1
Stoffel, W.2
-
118
-
-
0028100379
-
Topology of CNS myelin proteolipid protein: Evidence for the nonenzymatic glycosylation of extracytoplasmic domains in normal, and diabetic animals
-
Weimbs T. and Stoffel W. (1994) Topology of CNS myelin proteolipid protein: evidence for the nonenzymatic glycosylation of extracytoplasmic domains in normal, and diabetic animals. Biochemistry 33, 10408-10415.
-
(1994)
Biochemistry
, vol.33
, pp. 10408-10415
-
-
Weimbs, T.1
Stoffel, W.2
-
119
-
-
0020576951
-
Hypomyelinated mutant mice. V. Relationship between jp, and jpmsd re-examined on identical genetic backgrounds
-
Wolf M. K., Kardon G. B., Adcock L. H., and Billings-Gagliardi S. (1983) Hypomyelinated mutant mice. V. Relationship between jp, and jpmsd re-examined on identical genetic backgrounds. Brain Res. 271, 121-129.
-
(1983)
Brain Res.
, vol.271
, pp. 121-129
-
-
Wolf, M.K.1
Kardon, G.B.2
Adcock, L.H.3
Billings-Gagliardi, S.4
-
120
-
-
0030867942
-
gadd153/Chop10, a potential target gene of the transcriptional repressor ATF3
-
Wolfgang C. D., Chen B. P., Martindale J. L., Holbrook N. J., and Hai T. (1997) gadd153/Chop10, a potential target gene of the transcriptional repressor ATF3. Mol. Cell. Biol. 17, 6700-6707.
-
(1997)
Mol. Cell. Biol.
, vol.17
, pp. 6700-6707
-
-
Wolfgang, C.D.1
Chen, B.P.2
Martindale, J.L.3
Holbrook, N.J.4
Hai, T.5
-
122
-
-
0033559470
-
Proteolipid protein gene product can be secreted, and exhibit biological activity during early development
-
Yamada M., Ivanova A., Yamaguchi Y., Lees M. B., and Ikenaka K. (1999) Proteolipid protein gene product can be secreted, and exhibit biological activity during early development. J. Neurosci. 19, 2143-2151.
-
(1999)
J. Neurosci.
, vol.19
, pp. 2143-2151
-
-
Yamada, M.1
Ivanova, A.2
Yamaguchi, Y.3
Lees, M.B.4
Ikenaka, K.5
-
123
-
-
0034869755
-
Mutant Plp/DM20 cannot be processed to secrete PLP-related oligodendrocyte differentiation/survival factor
-
Yamada M., Jung M., Tetsushi K., et al. (2001) Mutant Plp/DM20 cannot be processed to secrete PLP-related oligodendrocyte differentiation/survival factor. Neurochem. Res. 26, 639-645.
-
(2001)
Neurochem. Res.
, vol.26
, pp. 639-645
-
-
Yamada, M.1
Jung, M.2
Tetsushi, K.3
-
124
-
-
0032477642
-
Jimpy (msd) mouse mutation, and connatal Pelizaeus-Merzbacher disease
-
Yamamoto T., Nanba E., Zhang H., et al. (1998) Jimpy (msd) mouse mutation, and connatal Pelizaeus-Merzbacher disease. Am. J. Med. Genet. 75, 439-440.
-
(1998)
Am. J. Med. Genet.
, vol.75
, pp. 439-440
-
-
Yamamoto, T.1
Nanba, E.2
Zhang, H.3
-
125
-
-
0034515724
-
ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs
-
Ye J., Rawson R. B., Komuro R., et al. (2000) ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs. Mol. Cell 6, 1355-1364.
-
(2000)
Mol. Cell
, vol.6
, pp. 1355-1364
-
-
Ye, J.1
Rawson, R.B.2
Komuro, R.3
-
126
-
-
0035957929
-
Activation of caspase-12, an endoplastic reticulum (ER) resident caspase, through tumor necrosis factor receptor-associated factor 2- dependent mechanism in response to the ER stress
-
Yoneda T., Imaizumi K., Oono K., et al. (2001) Activation of caspase-12, an endoplastic reticulum (ER) resident caspase, through tumor necrosis factor receptor-associated factor 2- dependent mechanism in response to the ER stress. J. Biol. Chem. 276, 13935-13940.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 13935-13940
-
-
Yoneda, T.1
Imaizumi, K.2
Oono, K.3
-
127
-
-
0032509216
-
Identification of the cis-acting endoplasmic reticulum stress response element responsible for transcriptional induction of mammalian glucose-regulated proteins. Involvement of basic leucine zipper transcription factors
-
Yoshida H., Haze K., Yanagi H., Yura T., and Mori K. (1998) Identification of the cis-acting endoplasmic reticulum stress response element responsible for transcriptional induction of mammalian glucose-regulated proteins. Involvement of basic leucine zipper transcription factors. J. Biol. Chem. 273, 33741-33749.
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 33741-33749
-
-
Yoshida, H.1
Haze, K.2
Yanagi, H.3
Yura, T.4
Mori, K.5
-
128
-
-
0026042012
-
Transgenic mouse model for central nervous system demyelination
-
Yoshioka T., Feigenbaum L., and Jay G. (1991) Transgenic mouse model for central nervous system demyelination. Mol. Cell. Biol. 11, 5479-5486.
-
(1991)
Mol. Cell. Biol.
, vol.11
, pp. 5479-5486
-
-
Yoshioka, T.1
Feigenbaum, L.2
Jay, G.3
-
129
-
-
0035929641
-
Homocysteine induces programmed cell death in human vascular endothelial cells through activation of the unfolded protein response
-
Zhang C., Cai Y., Adachi M. T., et al. (2001) Homocysteine induces programmed cell death in human vascular endothelial cells through activation of the unfolded protein response. J. Biol. Chem. 276, 35867-35874.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 35867-35874
-
-
Zhang, C.1
Cai, Y.2
Adachi, M.T.3
-
130
-
-
0037009521
-
A mitochondrial specific stress response in mammalian cells
-
Zhao Q., Wang J., Levichkin I. V., et al. (2002) A mitochondrial specific stress response in mammalian cells. EMBO J. 21, 4411-4419.
-
(2002)
EMBO J.
, vol.21
, pp. 4411-4419
-
-
Zhao, Q.1
Wang, J.2
Levichkin, I.V.3
-
131
-
-
0032054744
-
CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum
-
Zinszner H., Kuroda M., Wang X., et al. (1998) CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum. Genes Dev. 12, 982-995.
-
(1998)
Genes Dev.
, vol.12
, pp. 982-995
-
-
Zinszner, H.1
Kuroda, M.2
Wang, X.3
|