-
1
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
-
Group T.H.s.D.C.R. The Huntington's Disease Collaborative Research Group
-
Group, T.H.s.D.C.R. (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell, 72, 971-983.
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
2
-
-
0001589776
-
Differential loss of striatal projection neurons in Huntington disease
-
Reiner, A., Albin, R.L., Anderson, K.D., D'Amato, C.J., Penney, J.B. and Young, A.B. (1988) Differential loss of striatal projection neurons in Huntington disease. PNAS, 85, 5733-5737.
-
(1988)
PNAS
, vol.85
, pp. 5733-5737
-
-
Reiner, A.1
Albin, R.L.2
Anderson, K.D.3
D'Amato, C.J.4
Penney, J.B.5
Young, A.B.6
-
3
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale, D., Hackam, A., Wieczorek, A., Ellerby, L., Wellington, C., McCutcheon, K., Singaraja, R., Kazemi-Esfarjani, P., Devon, R., Kim, S.U. et al. (1998) Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat. Genet., 18, 150.
-
(1998)
Nat. Genet.
, vol.18
, pp. 150
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
Ellerby, L.4
Wellington, C.5
McCutcheon, K.6
Singaraja, R.7
Kazemi-Esfarjani, P.8
Devon, R.9
Kim, S.U.10
-
4
-
-
17144386565
-
cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues
-
Chiang, M.-C., Lee, Y.-C., Huang, C.-L. and Chern, Y. (2005) cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues. J. Biol. Chem., 280, 14331-14340.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 14331-14340
-
-
Chiang, M.-C.1
Lee, Y.-C.2
Huang, C.-L.3
Chern, Y.4
-
5
-
-
12944263711
-
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
-
Steffan, J.S., Kazantsev, A., Spasic-Boskovic, O., Greenwald, M., Zhu, Y.Z., Gohler, H., Wanker, E.E., Bates, G.P., Housman, D.E. and Thompson, L.M. (2000) The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA, 97, 6763-6768.
-
(2000)
Proc. Natl Acad. Sci. USA
, vol.97
, pp. 6763-6768
-
-
Steffan, J.S.1
Kazantsev, A.2
Spasic-Boskovic, O.3
Greenwald, M.4
Zhu, Y.Z.5
Gohler, H.6
Wanker, E.E.7
Bates, G.P.8
Housman, D.E.9
Thompson, L.M.10
-
6
-
-
0037418339
-
Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice
-
Duan, W., Guo, Z., Jiang, H., Ware, M., Li, X.-J. and Mattson, M.P. (2003) Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice. PNAS, 100, 2911-2916.
-
(2003)
PNAS
, vol.100
, pp. 2911-2916
-
-
Duan, W.1
Guo, Z.2
Jiang, H.3
Ware, M.4
Li, X.-J.5
Mattson, M.P.6
-
7
-
-
0015521813
-
Increased frequency of diabetes mellitus in patients with Huntington's chorea
-
Podolsky, S., Leopold, N.A. and Sax, D.S. (1972) Increased frequency of diabetes mellitus in patients with Huntington's chorea. Lancet, 1, 1356-1358.
-
(1972)
Lancet
, vol.1
, pp. 1356-1358
-
-
Podolsky, S.1
Leopold, N.A.2
Sax, D.S.3
-
8
-
-
0342368660
-
Proton magnetic resonance spectroscopy of cerebrospinal fluid in neurodegenerative disease: Indication of glial energy impairment in Huntington chorea, but not Parkinson disease
-
Garseth, M., Sonnewald, U., White, L.R., Rod, M., Zwart, J.A., Nygaard, O. and Aasly, J. (2000) Proton magnetic resonance spectroscopy of cerebrospinal fluid in neurodegenerative disease: Indication of glial energy impairment in Huntington chorea, but not Parkinson disease. J. Neurosci. Res., 60, 779-782.
-
(2000)
J. Neurosci. Res.
, vol.60
, pp. 779-782
-
-
Garseth, M.1
Sonnewald, U.2
White, L.R.3
Rod, M.4
Zwart, J.A.5
Nygaard, O.6
Aasly, J.7
-
9
-
-
0033009587
-
Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes
-
Hurlbert, M.S., Zhou, W., Wasmeier, C., Kaddis, F.G., Hutton, J.C. and Freed, C.R. (1999) Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes. Diabetes, 48, 649-651.
-
(1999)
Diabetes
, vol.48
, pp. 649-651
-
-
Hurlbert, M.S.1
Zhou, W.2
Wasmeier, C.3
Kaddis, F.G.4
Hutton, J.C.5
Freed, C.R.6
-
10
-
-
18144386929
-
Tryptophan metabolism and oxidative stress in patients with Huntington's disease
-
Stoy, N., Mackay, G.M., Forrest, C.M., Christofides, J., Egerton, M., Stone, T.W. and Darlington, L.G. (2005) Tryptophan metabolism and oxidative stress in patients with Huntington's disease. J. Neurochem., 93, 611-623.
-
(2005)
J. Neurochem.
, vol.93
, pp. 611-623
-
-
Stoy, N.1
Mackay, G.M.2
Forrest, C.M.3
Christofides, J.4
Egerton, M.5
Stone, T.W.6
Darlington, L.G.7
-
11
-
-
33645085291
-
Huntington disease patients and transgenic mice have similar pro-catabolic serum metabolite profiles
-
Underwood, B.R., Broadhurst, D., Dunn, W.B., Ellis, D.I., Michell, A.W., Vacher, C., Mosedale, D.E., Kell, D.B., Barker, R.A., Grainger, D.J. et al. (2006) Huntington disease patients and transgenic mice have similar pro-catabolic serum metabolite profiles. Brain, 129, 877-886.
-
(2006)
Brain
, vol.129
, pp. 877-886
-
-
Underwood, B.R.1
Broadhurst, D.2
Dunn, W.B.3
Ellis, D.I.4
Michell, A.W.5
Vacher, C.6
Mosedale, D.E.7
Kell, D.B.8
Barker, R.A.9
Grainger, D.J.10
-
12
-
-
1642633757
-
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
-
Tanaka, M., Machida, Y., Niu, S., Ikeda, T., Jana, N.R., Doi, H., Kurosawa, M., Nekooki, M. and Nukina, N. (2004) Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med., 10, 148.
-
(2004)
Nat. Med.
, vol.10
, pp. 148
-
-
Tanaka, M.1
Machida, Y.2
Niu, S.3
Ikeda, T.4
Jana, N.R.5
Doi, H.6
Kurosawa, M.7
Nekooki, M.8
Nukina, N.9
-
13
-
-
0032919205
-
Formation of polyglutamine inclusions in non-CNS tissue
-
Sathasivam, K., Hobbs, C., Turmaine, M., Mangiarini, L., Mahal, A., Bertaux, F., Wanker, E.E., Doherty, P., Davies, S.W. and Bates, G.P. (1999) Formation of polyglutamine inclusions in non-CNS tissue. Hum. Mol. Genet., 8, 813-822.
-
(1999)
Hum. Mol. Genet.
, vol.8
, pp. 813-822
-
-
Sathasivam, K.1
Hobbs, C.2
Turmaine, M.3
Mangiarini, L.4
Mahal, A.5
Bertaux, F.6
Wanker, E.E.7
Doherty, P.8
Davies, S.W.9
Bates, G.P.10
-
14
-
-
0022990082
-
Mammalian urea cycle enzymes
-
Jackson, M.J., Beaudet, A.L. and O'Brien, W.E. (1986) Mammalian urea cycle enzymes. Annu. Rev. Genet., 20, 431-464.
-
(1986)
Annu. Rev. Genet.
, vol.20
, pp. 431-464
-
-
Jackson, M.J.1
Beaudet, A.L.2
O'Brien, W.E.3
-
15
-
-
2642513241
-
Clinical manifestations of inborn errors of the urea cycle and related metabolic disorders during childhood
-
discussion 1630S-1632S, 1667S-1672S
-
Endo, F., Matsuura, T., Yanagita, K. and Matsuda, I. (2004) Clinical manifestations of inborn errors of the urea cycle and related metabolic disorders during childhood. J. Nutr., 134, 1605S-1609S, discussion 1630S-1632S, 1667S-1672S.
-
(2004)
J. Nutr.
, vol.134
-
-
Endo, F.1
Matsuura, T.2
Yanagita, K.3
Matsuda, I.4
-
16
-
-
0036172346
-
Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine
-
Karpuj, M.V., Becher, M.W., Springer, J.E., Chabas, D., Youssef, S., Pedotti, R., Mitchell, D. and Steinman, L. (2002) Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat. Med., 8, 143-149.
-
(2002)
Nat. Med.
, vol.8
, pp. 143-149
-
-
Karpuj, M.V.1
Becher, M.W.2
Springer, J.E.3
Chabas, D.4
Youssef, S.5
Pedotti, R.6
Mitchell, D.7
Steinman, L.8
-
17
-
-
20244362093
-
CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model
-
Chou, S.-Y., Lee, Y.-C., Chen, H.-M., Chiang, M.-C., Lai, H.-L., Chang, H.-H., Wu, Y.-C., Sun, C.-N., Chien, C.-L., Lin, Y.-S. et al. (2005) CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model. J. Neurochem., 93, 310-320.
-
(2005)
J. Neurochem.
, vol.93
, pp. 310-320
-
-
Chou, S.-Y.1
Lee, Y.-C.2
Chen, H.-M.3
Chiang, M.-C.4
Lai, H.-L.5
Chang, H.-H.6
Wu, Y.-C.7
Sun, C.-N.8
Chien, C.-L.9
Lin, Y.-S.10
-
18
-
-
34047116650
-
Recurrent psychosis associated with liver disease and elevated blood ammonia
-
Havens, L.L. and Child, C.G. (1955) Recurrent psychosis associated with liver disease and elevated blood ammonia. N. Engl. J. Med., 252, 756-759.
-
(1955)
N. Engl. J. Med.
, vol.252
, pp. 756-759
-
-
Havens, L.L.1
Child, C.G.2
-
19
-
-
0033976580
-
Huntington's disease and its association with psychopathology
-
De Marchi, N. and Mennella, R. (2000) Huntington's disease and its association with psychopathology. Harv. Rev. Psychiatry, 7, 278-289.
-
(2000)
Harv. Rev. Psychiatry
, vol.7
, pp. 278-289
-
-
De Marchi, N.1
Mennella, R.2
-
20
-
-
0035862896
-
Neurological abnormalities in a knock-in mouse model of Huntington's disease
-
Lin, C.-H., Tallaksen-Greene, S., Chien, W.-M., Cearley, J.A., Jackson, W.S., Crouse, A.B., Ren, S., Li, X.-J., Albin, R.L. and Detloff, P.J. (2001) Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum. Mol. Genet., 10, 137-144.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 137-144
-
-
Lin, C.-H.1
Tallaksen-Greene, S.2
Chien, W.-M.3
Cearley, J.A.4
Jackson, W.S.5
Crouse, A.B.6
Ren, S.7
Li, X.-J.8
Albin, R.L.9
Detloff, P.J.10
-
21
-
-
0035145675
-
The nutritional management of urea cycle disorders
-
discussion S44-S45
-
Leonard, J.V. (2001) The nutritional management of urea cycle disorders. J. Pediatr., 138, S40-S44, discussion S44-S45.
-
(2001)
J. Pediatr.
, vol.138
-
-
Leonard, J.V.1
-
22
-
-
0035919701
-
Loss of Huntingtin-mediated BDNF gene transcription in Huntington's disease
-
Zuccato, C., Ciammola, A., Rigamonti, D., Leavitt, B.R., Goffredo, D., Conti, L., MacDonald, M.E., Friedlander, R.M., Silani, V., Hayden, M.R. et al. (2001) Loss of Huntingtin-mediated BDNF gene transcription in Huntington's disease. Science, 293, 493-498.
-
(2001)
Science
, vol.293
, pp. 493-498
-
-
Zuccato, C.1
Ciammola, A.2
Rigamonti, D.3
Leavitt, B.R.4
Goffredo, D.5
Conti, L.6
MacDonald, M.E.7
Friedlander, R.M.8
Silani, V.9
Hayden, M.R.10
-
23
-
-
0036566675
-
Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin
-
Wyttenbach, A., Sauvageot, O., Carmichael, J., Diaz-Latoud, C., Arrigo, A.P. and Rubinsztein, D.C. (2002) Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin. Hum. Mol. Genet., 11, 1137-1151.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 1137-1151
-
-
Wyttenbach, A.1
Sauvageot, O.2
Carmichael, J.3
Diaz-Latoud, C.4
Arrigo, A.P.5
Rubinsztein, D.C.6
-
24
-
-
0034641589
-
Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
-
Jana, N.R., Tanaka, M., Wang, G. and Nukina, N. (2000) Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity. Hum. Mol. Genet., 9, 2009-2018.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2009-2018
-
-
Jana, N.R.1
Tanaka, M.2
Wang, G.3
Nukina, N.4
-
25
-
-
3242695184
-
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
-
Hay, D.G., Sathasivam, K., Tobaben, S., Stahl, B., Marber, M., Mestril, R., Mahal, A., Smith, D.L., Woodman, B. and Bates, G.P. (2004) Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Hum. Mol. Genet., 13, 1389-1405.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 1389-1405
-
-
Hay, D.G.1
Sathasivam, K.2
Tobaben, S.3
Stahl, B.4
Marber, M.5
Mestril, R.6
Mahal, A.7
Smith, D.L.8
Woodman, B.9
Bates, G.P.10
-
26
-
-
14844341251
-
Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila
-
Agrawal, N., Pallos, J., Slepko, N., Apostol, B.L., Bodai, L., Chang, L.W., Chiang, A.S., Thompson, L.M. and Marsh, J.L. (2005) Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila. Proc. Natl Acad. Sci. USA, 102, 3777-3781.
-
(2005)
Proc. Natl Acad. Sci. USA
, vol.102
, pp. 3777-3781
-
-
Agrawal, N.1
Pallos, J.2
Slepko, N.3
Apostol, B.L.4
Bodai, L.5
Chang, L.W.6
Chiang, A.S.7
Thompson, L.M.8
Marsh, J.L.9
-
27
-
-
0025744556
-
Induction of hsp 72/73 by herbimycin A, an inhibitor of transformation by tyrosine kinase oncogenes
-
Murakami, Y., Uehara, Y., Yamamoto, C., Fukazawa, H. and Mizuno, S. (1991) Induction of hsp 72/73 by herbimycin A, an inhibitor of transformation by tyrosine kinase oncogenes. Exp. Cell Res., 195, 338-344.
-
(1991)
Exp. Cell Res.
, vol.195
, pp. 338-344
-
-
Murakami, Y.1
Uehara, Y.2
Yamamoto, C.3
Fukazawa, H.4
Mizuno, S.5
-
28
-
-
0028051810
-
Rat argininosuccinate lyase promoter: The dyad-symmetric CCAAT box sequence CCAATTGG in the promoter is recognized by NF-Y
-
Matsubasa, T., Takiguchi, M., Matsuda, I. and Mori, M. (1994) Rat argininosuccinate lyase promoter: The dyad-symmetric CCAAT box sequence CCAATTGG in the promoter is recognized by NF-Y. J. Biochem.(Tokyo), 116, 1044-1055.
-
(1994)
J. Biochem.(Tokyo)
, vol.116
, pp. 1044-1055
-
-
Matsubasa, T.1
Takiguchi, M.2
Matsuda, I.3
Mori, M.4
-
29
-
-
0037101837
-
Polyglutamine and transcription: Gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
-
Luthi-Carter, R., Strand, A.D., Hanson, S.A., Kooperberg, C., Schilling, G., La Spada, A.R., Merry, D.E., Young, A.B., Ross, C.A., Borchelt, D.R. et al. (2002) Polyglutamine and transcription: Gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects. Hum. Mol. Genet., 11, 1927-1937.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 1927-1937
-
-
Luthi-Carter, R.1
Strand, A.D.2
Hanson, S.A.3
Kooperberg, C.4
Schilling, G.5
La Spada, A.R.6
Merry, D.E.7
Young, A.B.8
Ross, C.A.9
Borchelt, D.R.10
-
30
-
-
0032538639
-
Hypoglycemia-associated hyperammonemia caused by impaired expression of ornithine cycle enzyme genes in C/EBPalpha knockout mice
-
Kimura, T., Christoffels, V.M., Chowdhury, S., Iwase, K., Matsuzaki, H., Mori, M., Lamers, W.H., Darlington, G.J. and Takiguchi, M. (1998) Hypoglycemia-associated hyperammonemia caused by impaired expression of ornithine cycle enzyme genes in C/EBPalpha knockout mice. J. Biol. Chem., 273, 27505-27510.
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 27505-27510
-
-
Kimura, T.1
Christoffels, V.M.2
Chowdhury, S.3
Iwase, K.4
Matsuzaki, H.5
Mori, M.6
Lamers, W.H.7
Darlington, G.J.8
Takiguchi, M.9
-
31
-
-
0034141972
-
CREB-binding protein and p300: Molecular integrators of hematopoietic transcription
-
Blobel, G.A. (2000) CREB-binding protein and p300: Molecular integrators of hematopoietic transcription. Blood, 95, 745-755.
-
(2000)
Blood
, vol.95
, pp. 745-755
-
-
Blobel, G.A.1
-
32
-
-
0026029709
-
CCAAT/enhancer binding protein gene promoter: Binding of nuclear factors during differentiation of 3T3-L1 preadipocytes
-
Christy, R.J., Kaestner, K.H., Geiman, D.E. and Lane, M.D. (1991) CCAAT/ enhancer binding protein gene promoter: Binding of nuclear factors during differentiation of 3T3-L1 preadipocytes. Proc. Natl Acad. Sci. USA, 88, 2593-2597.
-
(1991)
Proc. Natl Acad. Sci. USA
, vol.88
, pp. 2593-2597
-
-
Christy, R.J.1
Kaestner, K.H.2
Geiman, D.E.3
Lane, M.D.4
-
33
-
-
0031460097
-
Superoxide production and antioxidant enzymes in ammonia intoxication in rats
-
Kosenko, E., Kaminsky, Y., Kaminsky, A., Valencia, M., Lee, L., Hermenegildo, C. and Felipo, V. (1997) Superoxide production and antioxidant enzymes in ammonia intoxication in rats. Free Radic. Res., 27, 637-644.
-
(1997)
Free Radic. Res.
, vol.27
, pp. 637-644
-
-
Kosenko, E.1
Kaminsky, Y.2
Kaminsky, A.3
Valencia, M.4
Lee, L.5
Hermenegildo, C.6
Felipo, V.7
-
34
-
-
33748577363
-
Selective neuronal degeneration in Huntington's disease
-
Cowan, C.M. and Raymond, L.A. (2006) Selective neuronal degeneration in Huntington's disease. Curr. Top. Dev. Biol., 75, 25-71.
-
(2006)
Curr. Top. Dev. Biol.
, vol.75
, pp. 25-71
-
-
Cowan, C.M.1
Raymond, L.A.2
-
35
-
-
0030863299
-
Nutrient deprivation of cultured rat hepatocytes increases the desferrioxamine-available iron pool and augments the sensitivity to hydrogen peroxide
-
Ollinger, K. and Roberg, K. (1997) Nutrient deprivation of cultured rat hepatocytes increases the desferrioxamine-available iron pool and augments the sensitivity to hydrogen peroxide. J. Biol. Chem., 272, 23707-23711.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 23707-23711
-
-
Ollinger, K.1
Roberg, K.2
-
36
-
-
0043204997
-
The anti-ageing effects of caloric restriction may involve stimulation of macroautophagy and lysosomal degradation, and can be intensified pharmacologically
-
Bergamini, E., Cavallini, G., Donati, A. and Gori, Z. (2003) The anti-ageing effects of caloric restriction may involve stimulation of macroautophagy and lysosomal degradation, and can be intensified pharmacologically. Biomed. Pharmacother., 57, 203-208.
-
(2003)
Biomed. Pharmacother.
, vol.57
, pp. 203-208
-
-
Bergamini, E.1
Cavallini, G.2
Donati, A.3
Gori, Z.4
-
37
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
Ravikumar, B., Vacher, C., Berger, Z., Davies, J.E., Luo, S., Oroz, L.G., Scaravilli, F., Easton, D.F., Duden, R., O'Kane, C.J. et al. (2004) Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet., 36, 585-595.
-
(2004)
Nat. Genet.
, vol.36
, pp. 585-595
-
-
Ravikumar, B.1
Vacher, C.2
Berger, Z.3
Davies, J.E.4
Luo, S.5
Oroz, L.G.6
Scaravilli, F.7
Easton, D.F.8
Duden, R.9
O'Kane, C.J.10
-
38
-
-
24944482408
-
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation
-
Iwata, A., Christianson, J.C., Bucci, M., Ellerby, L.M., Nukina, N., Forno, L.S. and Kopito, R.R. (2005) Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation. Proc. Natl Acad. Sci. USA, 102, 13135-13140.
-
(2005)
Proc. Natl Acad. Sci. USA
, vol.102
, pp. 13135-13140
-
-
Iwata, A.1
Christianson, J.C.2
Bucci, M.3
Ellerby, L.M.4
Nukina, N.5
Forno, L.S.6
Kopito, R.R.7
-
39
-
-
33644540193
-
Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway
-
Yamamoto, A., Cremona, M.L. and Rothman, J.E. (2006) Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway. J. Cell Biol., 172, 719-731.
-
(2006)
J. Cell Biol.
, vol.172
, pp. 719-731
-
-
Yamamoto, A.1
Cremona, M.L.2
Rothman, J.E.3
-
40
-
-
28744455086
-
Almost all about citrulline in mammals
-
Curis, E., Nicolis, I., Moinard, C., Osowska, S., Zerrouk, N., Benazeth, S. and Cynober, L. (2005) Almost all about citrulline in mammals. Amino acids, 29, 177-205.
-
(2005)
Amino Acids
, vol.29
, pp. 177-205
-
-
Curis, E.1
Nicolis, I.2
Moinard, C.3
Osowska, S.4
Zerrouk, N.5
Benazeth, S.6
Cynober, L.7
-
41
-
-
0035021811
-
Identification and characterization of the potential promoter regions of 1031 kinds of human genes
-
Suzuki, Y., Tsunoda, T., Sese, J., Taira, H., Mizushima-Sugano, J., Hata, H., Ota, T., Isogai, T., Tanaka, T., Nakamura, Y. et al. (2001) Identification and characterization of the potential promoter regions of 1031 kinds of human genes. Genome Res., 11, 677-684.
-
(2001)
Genome Res.
, vol.11
, pp. 677-684
-
-
Suzuki, Y.1
Tsunoda, T.2
Sese, J.3
Taira, H.4
Mizushima-Sugano, J.5
Hata, H.6
Ota, T.7
Isogai, T.8
Tanaka, T.9
Nakamura, Y.10
-
42
-
-
0033083803
-
Cross-regulation of C/EBP[alpha] and PPAR[gamma] controls the transcriptional pathway of adipogenesis and insulin sensitivity
-
Wu, Z., Rosen, E.D., Brun, R., Hauser, S., Adelmant, G., Troy, A.E., McKeon, C., Darlington, G.J. and Spiegelman, B.M. (1999) Cross-regulation of C/EBP[alpha] and PPAR[gamma] controls the transcriptional pathway of adipogenesis and insulin sensitivity. Mol. Cell, 3, 151.
-
(1999)
Mol. Cell
, vol.3
, pp. 151
-
-
Wu, Z.1
Rosen, E.D.2
Brun, R.3
Hauser, S.4
Adelmant, G.5
Troy, A.E.6
McKeon, C.7
Darlington, G.J.8
Spiegelman, B.M.9
-
43
-
-
0029160028
-
Impaired energy homeostasis in C/EBP alpha knockout mice
-
Wang, N.D., Finegold, M.J., Bradley, A., Ou, C.N., Abdelsayed, S.V., Wilde, M.D., Taylor, L.R., Wilson, D.R. and Darlington, G.J. (1995) Impaired energy homeostasis in C/EBP alpha knockout mice. Science, 269, 1108-1112.
-
(1995)
Science
, vol.269
, pp. 1108-1112
-
-
Wang, N.D.1
Finegold, M.J.2
Bradley, A.3
Ou, C.N.4
Abdelsayed, S.V.5
Wilde, M.D.6
Taylor, L.R.7
Wilson, D.R.8
Darlington, G.J.9
-
44
-
-
17144395919
-
Chromatin immunoprecipitation (ChIP) on chip experiments uncover a widespread distribution of NF-Y binding CCAAT sites outside of core promoters
-
Testa, A., Donati, G., Yan, P., Romani, F., Huang, T.H.M., Vigano, M.A. and Mantovani, R. (2005) Chromatin immunoprecipitation (ChIP) on chip experiments uncover a widespread distribution of NF-Y binding CCAAT sites outside of core promoters. J. Biol. Chem., 280, 13606-13615.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 13606-13615
-
-
Testa, A.1
Donati, G.2
Yan, P.3
Romani, F.4
Huang, T.H.M.5
Vigano, M.A.6
Mantovani, R.7
-
45
-
-
0033851770
-
Recent advances in Huntington's disease
-
Gutekunst, C.A., Norflus, F. and Hersch, S.M. (2000) Recent advances in Huntington's disease. Curr. Opin. Neurol., 13, 445-450.
-
(2000)
Curr. Opin. Neurol.
, vol.13
, pp. 445-450
-
-
Gutekunst, C.A.1
Norflus, F.2
Hersch, S.M.3
-
46
-
-
0036175623
-
Mitochondrial dysfunction in acute hyperammonemia
-
Felipo, V. and Butterworth, R.F. (2002) Mitochondrial dysfunction in acute hyperammonemia. Neurochem. Int., 40, 487-491.
-
(2002)
Neurochem. Int.
, vol.40
, pp. 487-491
-
-
Felipo, V.1
Butterworth, R.F.2
-
47
-
-
0026583030
-
Effect of ammonium ions on synaptic transmission in the mammalian central nervous system
-
Szerb, J.C. and Butterworth, R.F. (1992) Effect of ammonium ions on synaptic transmission in the mammalian central nervous system. Prog. Neurobiol., 39, 135-153.
-
(1992)
Prog. Neurobiol.
, vol.39
, pp. 135-153
-
-
Szerb, J.C.1
Butterworth, R.F.2
-
48
-
-
0028892620
-
Evidence of excitotoxicity in the brain of the ornithine carbamoyltransferase deficient sparse fur mouse
-
Robinson, M.B., Hopkins, K., Batshaw, M.L., McLaughlin, B.A., Heyes, M.P. and Oster-Granite, M.L. (1995) Evidence of excitotoxicity in the brain of the ornithine carbamoyltransferase deficient sparse fur mouse. Brain Res. Dev. Brain Res., 90, 35-44.
-
(1995)
Brain Res. Dev. Brain Res.
, vol.90
, pp. 35-44
-
-
Robinson, M.B.1
Hopkins, K.2
Batshaw, M.L.3
McLaughlin, B.A.4
Heyes, M.P.5
Oster-Granite, M.L.6
-
49
-
-
0037056392
-
Huntington's disease: A randomized, controlled trial using the NMDA-antagonist amantadine
-
Verhagen Metman, L., Morris, M.J., Farmer, C., Gillespie, M., Mosby, K., Wuu, J. and Chase, T.N. (2002) Huntington's disease: A randomized, controlled trial using the NMDA-antagonist amantadine. Neurology, 59, 694-699.
-
(2002)
Neurology
, vol.59
, pp. 694-699
-
-
Verhagen Metman, L.1
Morris, M.J.2
Farmer, C.3
Gillespie, M.4
Mosby, K.5
Wuu, J.6
Chase, T.N.7
-
50
-
-
0026512056
-
Acute ammonia toxicity is mediated by the NMDA type of glutamate receptors
-
Marcaida, G., Felipo, V., Hermenegildo, C., Minana, M.D. and Grisolia, S. (1992) Acute ammonia toxicity is mediated by the NMDA type of glutamate receptors. FEBS Lett., 296, 67-68.
-
(1992)
FEBS Lett.
, vol.296
, pp. 67-68
-
-
Marcaida, G.1
Felipo, V.2
Hermenegildo, C.3
Minana, M.D.4
Grisolia, S.5
-
51
-
-
0029910901
-
NMDA receptor antagonists prevent acute ammonia toxicity in mice
-
Hermenegildo, C., Marcaida, G., Montoliu, C., Grisolia, S., Minana, M.D. and Felipo, V. (1996) NMDA receptor antagonists prevent acute ammonia toxicity in mice. Neurochem. Res., 21, 1237-1244.
-
(1996)
Neurochem Res.
, vol.21
, pp. 1237-1244
-
-
Hermenegildo, C.1
Marcaida, G.2
Montoliu, C.3
Grisolia, S.4
Minana, M.D.5
Felipo, V.6
-
52
-
-
0036652765
-
Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease
-
Schiefer, J., Landwehrmeyer, G.B., Luesse, H.G., Sprunken, A., Puls, C., Milkereit, A., Milkereit, E. and Kosinski, C.M. (2002) Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease. Mov. Disord., 17, 748-757.
-
(2002)
Mov. Disord.
, vol.17
, pp. 748-757
-
-
Schiefer, J.1
Landwehrmeyer, G.B.2
Luesse, H.G.3
Sprunken, A.4
Puls, C.5
Milkereit, A.6
Milkereit, E.7
Kosinski, C.M.8
-
53
-
-
4444225612
-
Dietary intake of macronutrients, micronutrients, and other dietary constituents: United States 1988-94
-
Bialostosky, K., Wright, J.D., Kennedy-Stephenson, J., McDowell, M. and Johnson, C.L. (2002) Dietary intake of macronutrients, micronutrients, and other dietary constituents: United States 1988-94. Vital Health Stat., 11, 1-158.
-
(2002)
Vital Health Stat.
, vol.11
, pp. 1-158
-
-
Bialostosky, K.1
Wright, J.D.2
Kennedy-Stephenson, J.3
McDowell, M.4
Johnson, C.L.5
-
54
-
-
12144288251
-
Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset
-
Wexler, N.S., Lorimer, J., Porter, J., Gomez, F., Moskowitz, C., Shackell, E., Marder, K., Penchaszadeh, G., Roberts, S.A., Gayan, J. et al. (2004) Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc. Natl Acad. Sci. USA, 101, 3498-3503.
-
(2004)
Proc. Natl Acad. Sci. USA
, vol.101
, pp. 3498-3503
-
-
Wexler, N.S.1
Lorimer, J.2
Porter, J.3
Gomez, F.4
Moskowitz, C.5
Shackell, E.6
Marder, K.7
Penchaszadeh, G.8
Roberts, S.A.9
Gayan, J.10
-
55
-
-
0036756963
-
CAG mutation effect on rate of progression in Huntington's disease
-
Squitieri, F., Cannella, M. and Simonelli, M. (2002) CAG mutation effect on rate of progression in Huntington's disease. Neurol. Sci., 23 (Suppl. 2), S107-S108.
-
(2002)
Neurol. Sci.
, vol.23
, Issue.SUPPL. 2
-
-
Squitieri, F.1
Cannella, M.2
Simonelli, M.3
-
56
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S.W. et al. (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell, 87, 493-506.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
Cozens, B.4
Harper, A.5
Hetherington, C.6
Lawton, M.7
Trottier, Y.8
Lehrach, H.9
Davies, S.W.10
-
57
-
-
85047690932
-
ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease
-
Wu, J.Y., Kao, H.J., Li, S.C., Stevens, R., Hillman, S., Millington, D. and Chen, Y.T. (2004) ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease. J. Clin. Invest., 113, 434-440.
-
(2004)
J. Clin. Invest.
, vol.113
, pp. 434-440
-
-
Wu, J.Y.1
Kao, H.J.2
Li, S.C.3
Stevens, R.4
Hillman, S.5
Millington, D.6
Chen, Y.T.7
-
58
-
-
0034878063
-
A pilot study of neonatal screening by electrospray ionization tandem mass spectrometry in Taiwan
-
Lin, W.D., Wu, J.Y., Lai, C.C., Tsai, F.J., Tsai, C.H., Lin, S.P. and Niu, D.M. (2001) A pilot study of neonatal screening by electrospray ionization tandem mass spectrometry in Taiwan. Acta Paediatr. Taiwan, 42, 224-230.
-
(2001)
Acta Paediatr. Taiwan
, vol.42
, pp. 224-230
-
-
Lin, W.D.1
Wu, J.Y.2
Lai, C.C.3
Tsai, F.J.4
Tsai, C.H.5
Lin, S.P.6
Niu, D.M.7
-
59
-
-
0034702030
-
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
-
Luthi-Carter, R., Strand, A., Peters, N.L., Solano, S.M., Hollingsworth, Z.R., Menon, A.S., Frey, A.S., Spektor, B.S., Penney, E.B., Schilling, G. et al. (2000) Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum. Mol. Genet., 9, 1259-1271.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 1259-1271
-
-
Luthi-Carter, R.1
Strand, A.2
Peters, N.L.3
Solano, S.M.4
Hollingsworth, Z.R.5
Menon, A.S.6
Frey, A.S.7
Spektor, B.S.8
Penney, E.B.9
Schilling, G.10
-
60
-
-
33646421164
-
Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase
-
Borrell-Pages, M., Canals, J.M., Cordelieres, F.P., Parker, J.A., Pineda, J.R., Grange, G., Bryson, E.A., Guillermier, M., Hirsch, E., Hantraye, P. et al. (2006) Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J. Clin. Invest., 116, 1410-1424.
-
(2006)
J. Clin. Invest.
, vol.116
, pp. 1410-1424
-
-
Borrell-Pages, M.1
Canals, J.M.2
Cordelieres, F.P.3
Parker, J.A.4
Pineda, J.R.5
Grange, G.6
Bryson, E.A.7
Guillermier, M.8
Hirsch, E.9
Hantraye, P.10
-
61
-
-
0032566719
-
Expression of type VI adenylyl cyclase in the central nervous system: Implication for a potential regulator of multiple signals in different neurotransmitter systems
-
Liu, F.-C., Wu, G.-C., Hsieh, S.-T., Lai, H.-L., Wang, H.-F., Wang, T.-W. and Chern, Y. (1998) Expression of type VI adenylyl cyclase in the central nervous system: Implication for a potential regulator of multiple signals in different neurotransmitter systems. FEBS Lett., 436, 92-98.
-
(1998)
FEBS Lett.
, vol.436
, pp. 92-98
-
-
Liu, F.-C.1
Wu, G.-C.2
Hsieh, S.-T.3
Lai, H.-L.4
Wang, H.-F.5
Wang, T.-W.6
Chern, Y.7
-
62
-
-
0242624564
-
Characterization of the rat A2A adenosine receptor gene: A 4.8-kb promoter-proximal DNA fragment confers selective expression in the central nervous system
-
Lee, Y.C., Chien, C.L., Sun, C.N., Huang, C.L., Huang, N.K., Chiang, M.C., Lai, H.L., Lin, Y.S., Chou, S.Y., Wang, C.K. et al. (2003) Characterization of the rat A2A adenosine receptor gene: A 4.8-kb promoter-proximal DNA fragment confers selective expression in the central nervous system. Eur. J. Neurosci., 18, 1786-1796.
-
(2003)
Eur. J. Neurosci.
, vol.18
, pp. 1786-1796
-
-
Lee, Y.C.1
Chien, C.L.2
Sun, C.N.3
Huang, C.L.4
Huang, N.K.5
Chiang, M.C.6
Lai, H.L.7
Lin, Y.S.8
Chou, S.Y.9
Wang, C.K.10
-
63
-
-
0035951109
-
Mechanisms for intragenic complementation at the human argininosuccinate lyase locus
-
Yu, B., Thompson, G.D., Yip, P., Howell, P.L. and Davidson, A.R. (2001) Mechanisms for intragenic complementation at the human argininosuccinate lyase locus. Biochemistry, 40, 15581-15590.
-
(2001)
Biochemistry
, vol.40
, pp. 15581-15590
-
-
Yu, B.1
Thompson, G.D.2
Yip, P.3
Howell, P.L.4
Davidson, A.R.5
-
64
-
-
4344662343
-
Argininosuccinate synthetase is reversibly inactivated by S-nitrosylation in vitro and in vivo
-
Hao, G., Xie, L. and Gross, S.S. (2004) Argininosuccinate synthetase is reversibly inactivated by S-nitrosylation in vitro and in vivo. J. Biol. Chem., 279, 36192-36200.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 36192-36200
-
-
Hao, G.1
Xie, L.2
Gross, S.S.3
-
65
-
-
0031051521
-
Protein kinase C inhibits adenylyl cyclase type VI activity during desensitization of the A2a-adenosine receptor-mediated cAMP response
-
Lai, H.L., Yang, T.H., Messing, R.O., Ching, Y.H., Lin, S.C. and Chern, Y. (1997) Protein kinase C inhibits adenylyl cyclase type VI activity during desensitization of the A2a-adenosine receptor-mediated cAMP response. J. Biol. Chem., 272, 4970-4977.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 4970-4977
-
-
Lai, H.L.1
Yang, T.H.2
Messing, R.O.3
Ching, Y.H.4
Lin, S.C.5
Chern, Y.6
-
66
-
-
0032879437
-
Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates
-
Wanker, E.E., Scherzinger, E., Heiser, V., Sittler, A., Eickhoff, H. and Lehrach, H. (1999) Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates. Meth. Enzymol., 309, 375-386.
-
(1999)
Meth. Enzymol.
, vol.309
, pp. 375-386
-
-
Wanker, E.E.1
Scherzinger, E.2
Heiser, V.3
Sittler, A.4
Eickhoff, H.5
Lehrach, H.6
|