-
1
-
-
0037160782
-
The muscular dystrophies
-
Emery AEH. The muscular dystrophies. Lancet 2002; 359: 687-695.
-
(2002)
Lancet
, vol.359
, pp. 687-695
-
-
Emery, A.E.H.1
-
2
-
-
0023614188
-
Dystrophin: the protein product of the Duchenne muscular dystrophy locus
-
Hoffman EP, Brown J, Robert H., Kunkel LM. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell 1987; 51: 919-928.
-
(1987)
Cell
, vol.51
, pp. 919-928
-
-
Hoffman, E.P.1
Brown, J.2
Robert, H.3
Kunkel, L.M.4
-
4
-
-
0035037179
-
Protein family review: dystrophins and dystrobrevins
-
reviews
-
Roberts RG. Protein family review: dystrophins and dystrobrevins. Gen Biol 2001; 2:reviews 3006.3001-3006.3007.
-
(2001)
Gen Biol
, vol.2
, pp. 30063001-30063007
-
-
Roberts, R.G.1
-
5
-
-
33846271135
-
Dystrophin, its interactions with other proteins, and implications
-
Ervasti JM. Dystrophin, its interactions with other proteins, and implications. Biochim Biophys Acta 2007; 1772: 108-117.
-
(2007)
Biochim Biophys Acta
, vol.1772
, pp. 108-117
-
-
Ervasti, J.M.1
-
7
-
-
0028303798
-
Searching for the 1 in 2,400,000: a review of dystrophin gene point mutations
-
Roberts RG, Gardner RJ, Bobrow M. Searching for the 1 in 2, 400, 000: a review of dystrophin gene point mutations. Hum Mutat 1994; 4: 1-11.
-
(1994)
Hum Mutat
, vol.4
, pp. 1-11
-
-
Roberts, R.G.1
Gardner, R.J.2
Bobrow, M.3
-
8
-
-
0029073192
-
Spectrum of small mutations in the dystrophin coding region
-
Prior TW, Bartolo C, Pearl DK, Papp AC, Snyder PJ, Sedra MS, Burghes AHM, Mendell JR. Spectrum of small mutations in the dystrophin coding region. Am J Hum Genet 1995; 57: 22-33.
-
(1995)
Am J Hum Genet
, vol.57
, pp. 22-33
-
-
Prior, T.W.1
Bartolo, C.2
Pearl, D.K.3
Papp, A.C.4
Snyder, P.J.5
Sedra, M.S.6
Burghes, A.H.M.7
Mendell, J.R.8
-
9
-
-
0030817080
-
Novel point mutations in the dystrophin gene
-
Sitnik R, Campiotto S, Vainzof M, Pavanello RC, Takata RI, Zatz M, Passos-Bueno MR. Novel point mutations in the dystrophin gene. Human Mutat 1997; 10: 217-222.
-
(1997)
Human Mutat
, vol.10
, pp. 217-222
-
-
Sitnik, R.1
Campiotto, S.2
Vainzof, M.3
Pavanello, R.C.4
Takata, R.I.5
Zatz, M.6
Passos-Bueno, M.R.7
-
10
-
-
0344420060
-
Dystrophin and mutations: One gene, several proteins, multiple phenotypes
-
Muntoni F, Torelli S, Ferlini A. Dystrophin and mutations: One gene, several proteins, multiple phenotypes. Lancet Neurol 2003; 2: 731-740.
-
(2003)
Lancet Neurol
, vol.2
, pp. 731-740
-
-
Muntoni, F.1
Torelli, S.2
Ferlini, A.3
-
11
-
-
33746766278
-
Entries in the Leiden Duchenne muscular dystrophy mutation database: an overview of mutation types and paradoxical cases that confirm the reading-frame rule
-
Aaartsma-Rus A, van Deutekom JCT, Fokkema IF, van Ommen G-JB, Den Dennen JT. Entries in the Leiden Duchenne muscular dystrophy mutation database: an overview of mutation types and paradoxical cases that confirm the reading-frame rule. Muscle Nerve 2006; 34: 135-144.
-
(2006)
Muscle Nerve
, vol.34
, pp. 135-144
-
-
Aaartsma-Rus, A.1
van Deutekom, J.C.T.2
Fokkema, I.F.3
van Ommen, G.-J.4
Den Dennen, J.T.5
-
12
-
-
0024538824
-
An autosomal transcript in skeletal muscle with homology to dystrophin
-
Love DR, Hill DF, Dickson G, Spurr NK, Byth BC, Marsden RF, Walsh FS, Edwards YH, Davies KE. An autosomal transcript in skeletal muscle with homology to dystrophin. Nature 1989; 339: 55-58.
-
(1989)
Nature
, vol.339
, pp. 55-58
-
-
Love, D.R.1
Hill, D.F.2
Dickson, G.3
Spurr, N.K.4
Byth, B.C.5
Marsden, R.F.6
Walsh, F.S.7
Edwards, Y.H.8
Davies, K.E.9
-
13
-
-
0025167294
-
Identification of a chromosome 6-encoded dystrophin-related protein
-
Khurana TS, Hoffman EP, Kunkel L. Identification of a chromosome 6-encoded dystrophin-related protein. J Biol Chem 1990; 265: 16717-16720.
-
(1990)
J Biol Chem
, vol.265
, pp. 16717-16720
-
-
Khurana, T.S.1
Hoffman, E.P.2
Kunkel, L.3
-
14
-
-
0026621049
-
Primary structure of dystrophin-related protein
-
Tinsley JM, Blake DJ, Roche A, Fairbrother U, Riss J, Byth BC, Knight AE, Kendrick-Jones J, Suthers GK, Love DR, Edwards YH, Davies KE. Primary structure of dystrophin-related protein. Nature 1992; 360: 591-593.
-
(1992)
Nature
, vol.360
, pp. 591-593
-
-
Tinsley, J.M.1
Blake, D.J.2
Roche, A.3
Fairbrother, U.4
Riss, J.5
Byth, B.C.6
Knight, A.E.7
Kendrick-Jones, J.8
Suthers, G.K.9
Love, D.R.10
Edwards, Y.H.11
Davies, K.E.12
-
15
-
-
0036087342
-
Function and genetics of dystrophin and dystrophin-related proteins in muscle
-
Blake DJ, Weir A, Newey SE, Davies KE. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev 2002; 82: 291-329.
-
(2002)
Physiol Rev
, vol.82
, pp. 291-329
-
-
Blake, D.J.1
Weir, A.2
Newey, S.E.3
Davies, K.E.4
-
16
-
-
0031727771
-
Expression of full-length utrophin prevents muscular dystrophy in mdx mice
-
Tinsley J, Deconinck N, Fisher R, Kahn D, Phelps S, Gillis J-M, Davies K. Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Nature Med 1998; 4: 1441-1444.
-
(1998)
Nature Med
, vol.4
, pp. 1441-1444
-
-
Tinsley, J.1
Deconinck, N.2
Fisher, R.3
Kahn, D.4
Phelps, S.5
Gillis, J.-M.6
Davies, K.7
-
17
-
-
0031775820
-
Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice
-
Rafael JA, Tinsley JM, Potter AC, Deconinck AE, Davies KE. Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice. Nat Genet 1998; 19: 79-82.
-
(1998)
Nat Genet
, vol.19
, pp. 79-82
-
-
Rafael, J.A.1
Tinsley, J.M.2
Potter, A.C.3
Deconinck, A.E.4
Davies, K.E.5
-
18
-
-
0036796262
-
Gene therapy of muscular dystrophy
-
Chamberlain JS. Gene therapy of muscular dystrophy. Hum Mol Genet 2002; 11: 2355-2362.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2355-2362
-
-
Chamberlain, J.S.1
-
19
-
-
0141594934
-
Advances in Duchenne muscular dystrophy gene therapy
-
van Deutekom JCT, van Ommen G-JB. Advances in Duchenne muscular dystrophy gene therapy. Nat Rev Genet 2003; 4: 774-783.
-
(2003)
Nat Rev Genet
, vol.4
, pp. 774-783
-
-
van Deutekom, J.C.T.1
van Ommen, G.-J.2
-
20
-
-
33644975603
-
Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: How close are we?
-
Miura P, Jasmin BJ. Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: How close are we? Trends Mol Med 2006; 12: 122-129.
-
(2006)
Trends Mol Med
, vol.12
, pp. 122-129
-
-
Miura, P.1
Jasmin, B.J.2
-
21
-
-
34548761362
-
Successful compensation for dystrophin deficiency by a helper-dependent adenovirus expressing full-length utrophin
-
Deol JR, Danialou G, Larochelle N, Bourget M, Moon J-S, Liu A-B, Gilbert R, Petrof BJ, Nalbantoglu J, Karpati G. Successful compensation for dystrophin deficiency by a helper-dependent adenovirus expressing full-length utrophin. Mol Ther 2007; 15: 1767-1774.
-
(2007)
Mol Ther
, vol.15
, pp. 1767-1774
-
-
Deol, J.R.1
Danialou, G.2
Larochelle, N.3
Bourget, M.4
Moon, J.-S.5
Liu, A.-B.6
Gilbert, R.7
Petrof, B.J.8
Nalbantoglu, J.9
Karpati, G.10
-
22
-
-
50549098032
-
Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/utrophin-deficient mice
-
Odom GL, Gregorevic P, Allen JM, Finn E, Chamberlain JS. Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/utrophin-deficient mice. Mol Ther 2008; 16: 1539-1545.
-
(2008)
Mol Ther
, vol.16
, pp. 1539-1545
-
-
Odom, G.L.1
Gregorevic, P.2
Allen, J.M.3
Finn, E.4
Chamberlain, J.S.5
-
23
-
-
66349121942
-
Functional substitution by TAT-utrophin in dystrophin-deficient mice
-
Sonnemann KJ, Heun-Johnson H, Turner AJ, Baltgalvis KA, Lowe DA, Ervasti JM. Functional substitution by TAT-utrophin in dystrophin-deficient mice. PLoS Med 2009; 6: e1000083.
-
(2009)
PLoS Med
, vol.6
-
-
Sonnemann, K.J.1
Heun-Johnson, H.2
Turner, A.J.3
Baltgalvis, K.A.4
Lowe, D.A.5
Ervasti, J.M.6
-
24
-
-
0025932274
-
Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle
-
Khurana TS, Watkins SC, Chafey P, Chelly J, Tome FMS, Fardeau M, Kaplan J-C, Kunkel LM. Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle. Neuromusc Disord 1991; 1: 185-194.
-
(1991)
Neuromusc Disord
, vol.1
, pp. 185-194
-
-
Khurana, T.S.1
Watkins, S.C.2
Chafey, P.3
Chelly, J.4
Tome, F.M.S.5
Fardeau, M.6
Kaplan, J.-C.7
Kunkel, L.M.8
-
25
-
-
0026344689
-
Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse
-
Love DR, Morris GE, Ellis JM, Fairbrother U, Marsden RF, Bloomfield JF, Edwards YH, Slater CP, Parry DJ, Davies KE. Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse. Proc Natl Acad Sci USA 1991; 88: 3243-3247.
-
(1991)
Proc Natl Acad Sci USA
, vol.88
, pp. 3243-3247
-
-
Love, D.R.1
Morris, G.E.2
Ellis, J.M.3
Fairbrother, U.4
Marsden, R.F.5
Bloomfield, J.F.6
Edwards, Y.H.7
Slater, C.P.8
Parry, D.J.9
Davies, K.E.10
-
26
-
-
0026460270
-
The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibers in dystrophies and inflammatory myopathies
-
Helliwell TR, Man NT, Morris GE, Davies KE. The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibers in dystrophies and inflammatory myopathies. Neuromusc Disord 1992; 2: 177-184.
-
(1992)
Neuromusc Disord
, vol.2
, pp. 177-184
-
-
Helliwell, T.R.1
Man, N.T.2
Morris, G.E.3
Davies, K.E.4
-
27
-
-
0029921129
-
Utrophin: a structural and functional comparison to dystrophin
-
Blake DJ, Tinsley JM, Davies KE. Utrophin: a structural and functional comparison to dystrophin. Brain Pathol 1996; 6: 37-47.
-
(1996)
Brain Pathol
, vol.6
, pp. 37-47
-
-
Blake, D.J.1
Tinsley, J.M.2
Davies, K.E.3
-
28
-
-
0026094250
-
Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle
-
Ohlendieck K, Ervasti JM, Matsumura K, Kahl SD, Leveille CJ, Campbell KP. Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle. Neuron 1991; 7: 499-508.
-
(1991)
Neuron
, vol.7
, pp. 499-508
-
-
Ohlendieck, K.1
Ervasti, J.M.2
Matsumura, K.3
Kahl, S.D.4
Leveille, C.J.5
Campbell, K.P.6
-
29
-
-
0031239725
-
Duchenne muscular dystrophy and the neuromuscular junction: the utrophin link
-
Gramolini AO, Jasmin BJ. Duchenne muscular dystrophy and the neuromuscular junction: the utrophin link. BioEssays 1997; 19: 747-750.
-
(1997)
BioEssays
, vol.19
, pp. 747-750
-
-
Gramolini, A.O.1
Jasmin, B.J.2
-
30
-
-
0033918661
-
Biochemical characterization of the actin-binding properties of utrophin
-
Moores CA, Kendrick-Jones J. Biochemical characterization of the actin-binding properties of utrophin. Cell Motil Cytoskeleton 2000; 46: 116-128.
-
(2000)
Cell Motil Cytoskeleton
, vol.46
, pp. 116-128
-
-
Moores, C.A.1
Kendrick-Jones, J.2
-
31
-
-
0025728005
-
A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice: immunological evidence
-
Pons F, Augier N, Léger JOC, Robert A, Tomé FMS, Fardeau M, Voit T, Nicholson LVB, Mornet D, Léger JJ. A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice: immunological evidence. FEBS Lett 1991; 282: 161-165.
-
(1991)
FEBS Lett
, vol.282
, pp. 161-165
-
-
Pons, F.1
Augier, N.2
Léger, J.O.C.3
Robert, A.4
Tomé, F.M.S.5
Fardeau, M.6
Voit, T.7
Nicholson, L.V.B.8
Mornet, D.9
Léger, J.J.10
-
32
-
-
0026621608
-
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
-
Matsumura K, Ervasti JM, Ohlendieck K, Kahl SD, Campbell KP. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 1992; 360: 588-591.
-
(1992)
Nature
, vol.360
, pp. 588-591
-
-
Matsumura, K.1
Ervasti, J.M.2
Ohlendieck, K.3
Kahl, S.D.4
Campbell, K.P.5
-
33
-
-
0028833396
-
Utrophin actin binding domain: analysis of actin binding and cellular targeting
-
Winder SJ, Hemmings L, Maciver SK, Bolton SJ, Tinsley JM, Davies KE, Critchley DR, Kendrick-Jones J. Utrophin actin binding domain: analysis of actin binding and cellular targeting. J Cell Sci 1995; 108: 63-71.
-
(1995)
J Cell Sci
, vol.108
, pp. 63-71
-
-
Winder, S.J.1
Hemmings, L.2
Maciver, S.K.3
Bolton, S.J.4
Tinsley, J.M.5
Davies, K.E.6
Critchley, D.R.7
Kendrick-Jones, J.8
-
34
-
-
0035999982
-
Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle
-
Rybakova IN, Patel JR, Davies KE, Yurchenco PD, Ervasti JM. Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle. Mol Biol Cell 2002; 13: 1512-1521.
-
(2002)
Mol Biol Cell
, vol.13
, pp. 1512-1521
-
-
Rybakova, I.N.1
Patel, J.R.2
Davies, K.E.3
Yurchenco, P.D.4
Ervasti, J.M.5
-
35
-
-
1842429177
-
ZZ domain is essentially required for the physiological binding of dystrophin and utrophin to β-dystroglycan
-
Ishikawa-Sakurai M, Yoshida M, Imamura M, Davies KE, Ozawa E. ZZ domain is essentially required for the physiological binding of dystrophin and utrophin to β-dystroglycan. Hum Mol Genet 2004; 13: 693-702.
-
(2004)
Hum Mol Genet
, vol.13
, pp. 693-702
-
-
Ishikawa-Sakurai, M.1
Yoshida, M.2
Imamura, M.3
Davies, K.E.4
Ozawa, E.5
-
38
-
-
12244272395
-
Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system
-
Squire S, Raymackers JM, Vandebrouck C, Potter A, Tinsley J, Fisher R, Gillis JM, Davies KE. Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system. Hum Mol Genet 2002; 11: 3333-3344.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 3333-3344
-
-
Squire, S.1
Raymackers, J.M.2
Vandebrouck, C.3
Potter, A.4
Tinsley, J.5
Fisher, R.6
Gillis, J.M.7
Davies, K.E.8
-
39
-
-
0034641893
-
Mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for Duchenne muscular dystrophy
-
Ahmad A, Brinson M, Hodges BL, Chamberlain JS, Amalfitano A. Mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for Duchenne muscular dystrophy. Hum Mol Genet 2000; 9: 2507-2515.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 2507-2515
-
-
Ahmad, A.1
Brinson, M.2
Hodges, B.L.3
Chamberlain, J.S.4
Amalfitano, A.5
-
40
-
-
0033572690
-
Crystal structure of the actin-binding region of utrophin reveals a head-to-tail dimer
-
Keep NH, Winder SJ, Moores CA, Walke S, Norwood FLM, Kendrick-Jones J. Crystal structure of the actin-binding region of utrophin reveals a head-to-tail dimer. Structure 1999; 7: 1539-1546.
-
(1999)
Structure
, vol.7
, pp. 1539-1546
-
-
Keep, N.H.1
Winder, S.J.2
Moores, C.A.3
Walke, S.4
Norwood, F.L.M.5
Kendrick-Jones, J.6
-
41
-
-
0034657791
-
The structure of the N-terminal actin-binding domain of human dystrophin and how mutations in this domain may cause Duchenne or Becker muscular dystrophy
-
Norwood FL, Sutherland-Smith AJ, Keep NH, Kendrick-Jones J. The structure of the N-terminal actin-binding domain of human dystrophin and how mutations in this domain may cause Duchenne or Becker muscular dystrophy. Structure 2000; 8: 481-491.
-
(2000)
Structure
, vol.8
, pp. 481-491
-
-
Norwood, F.L.1
Sutherland-Smith, A.J.2
Keep, N.H.3
Kendrick-Jones, J.4
-
42
-
-
77956994292
-
Missense mutations in dystrophin that trigger muscular dystrophy decrease protein stability and lead to cross-β aggregates
-
Singh SM, Kongari N, Cabello-Villegas J, Mallela KMG. Missense mutations in dystrophin that trigger muscular dystrophy decrease protein stability and lead to cross-β aggregates. Proc Natl Acad Sci USA 2010; 107: 15069-15074.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 15069-15074
-
-
Singh, S.M.1
Kongari, N.2
Cabello-Villegas, J.3
Mallela, K.M.G.4
-
43
-
-
77953108170
-
Disease-causing missense mutations in actin binding domain 1 of dystrophin induce thermodynamic instability and protein aggregation
-
Henderson DM, Lee A, Ervasti JM. Disease-causing missense mutations in actin binding domain 1 of dystrophin induce thermodynamic instability and protein aggregation. Proc Natl Acad Sci USA 2010; 107: 9632-9637.
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 9632-9637
-
-
Henderson, D.M.1
Lee, A.2
Ervasti, J.M.3
-
44
-
-
0029122523
-
Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice
-
Phelps SF, Hauser MA, Cole NM, Rafael JA, Hinkle RT, Faulkner JA, Chamberlain JS. Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice. Hum Mol Genet 1995; 4: 1251-1258.
-
(1995)
Hum Mol Genet
, vol.4
, pp. 1251-1258
-
-
Phelps, S.F.1
Hauser, M.A.2
Cole, N.M.3
Rafael, J.A.4
Hinkle, R.T.5
Faulkner, J.A.6
Chamberlain, J.S.7
-
45
-
-
0029906168
-
Amelioration of the dystrophin phenotype of mdx mice using a truncated utrophin transgene
-
Tinsley JM, Potter AC, Phelps SR, Fisher R, Trickett JI, Davies KE. Amelioration of the dystrophin phenotype of mdx mice using a truncated utrophin transgene. Nature 1996: 349-353.
-
(1996)
Nature
, pp. 349-353
-
-
Tinsley, J.M.1
Potter, A.C.2
Phelps, S.R.3
Fisher, R.4
Trickett, J.I.5
Davies, K.E.6
-
46
-
-
0030723508
-
Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice
-
Deconinck N, Tinsley J, Backer FD, Fisher R, Kahn D, Phelps S, Davies K, Gillis J-M. Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice. Nat Med 1997; 3: 1216-1221.
-
(1997)
Nat Med
, vol.3
, pp. 1216-1221
-
-
Deconinck, N.1
Tinsley, J.2
Backer, F.D.3
Fisher, R.4
Kahn, D.5
Phelps, S.6
Davies, K.7
Gillis, J.-M.8
-
47
-
-
0033587139
-
Adenovirus-mediated utrophin gene transfer mitigates the dystrophin phenotype of mdx mouse muscles
-
Gilbert R, Nalbantoglu J, Petrof BJ, Ebihara S, Guibinga GH, Tinsley JM, Kamen A, Massie B, Davies KE, Karpati G. Adenovirus-mediated utrophin gene transfer mitigates the dystrophin phenotype of mdx mouse muscles. Hum Gene Ther 1999; 10: 1299-1310.
-
(1999)
Hum Gene Ther
, vol.10
, pp. 1299-1310
-
-
Gilbert, R.1
Nalbantoglu, J.2
Petrof, B.J.3
Ebihara, S.4
Guibinga, G.H.5
Tinsley, J.M.6
Kamen, A.7
Massie, B.8
Davies, K.E.9
Karpati, G.10
-
48
-
-
0033994291
-
Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene
-
Wakefield PM, Tinsley JM, Wood MJA, Gilbert R, Karpati G, Davies KE. Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene. Gene Therapy 2000; 7: 201-204.
-
(2000)
Gene Therapy
, vol.7
, pp. 201-204
-
-
Wakefield, P.M.1
Tinsley, J.M.2
Wood, M.J.A.3
Gilbert, R.4
Karpati, G.5
Davies, K.E.6
-
49
-
-
0034610364
-
Adeno-associated virus vector carrying human minidystrophin genes effectively ameliorates muscular dystrophy in mdx mouse model
-
Wang B, Li J, Xiao X. Adeno-associated virus vector carrying human minidystrophin genes effectively ameliorates muscular dystrophy in mdx mouse model. Proc Natl Acad Sci USA 2000; 97: 13714-13719.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 13714-13719
-
-
Wang, B.1
Li, J.2
Xiao, X.3
-
50
-
-
33745894322
-
rAAV6-microdystrophin preserves muscle function and extends lifespan in severely dystrophic mice
-
Gregorevic P, Allen JM, Minami E, Blankenship MJ, Haraguchi M, Meuse L, Finn E, Adams ME, Froehner SC, Murry CE, Chamberlain JS. rAAV6-microdystrophin preserves muscle function and extends lifespan in severely dystrophic mice. Nat Med 2006; 12: 787-789.
-
(2006)
Nat Med
, vol.12
, pp. 787-789
-
-
Gregorevic, P.1
Allen, J.M.2
Minami, E.3
Blankenship, M.J.4
Haraguchi, M.5
Meuse, L.6
Finn, E.7
Adams, M.E.8
Froehner, S.C.9
Murry, C.E.10
Chamberlain, J.S.11
-
51
-
-
0036823730
-
Viral vectors for gene transfer of micro-, mini-, or full-length dystrophin
-
Scott JM, Li S, harper SQ. Viral vectors for gene transfer of micro-, mini-, or full-length dystrophin. Neuromuscular Disord 2002; 12: s23-s29.
-
(2002)
Neuromuscular Disord
, vol.12
-
-
Scott, J.M.1
Li, S.2
Harper, S.Q.3
-
52
-
-
0042170127
-
Gene therapy for muscular dystrophy-a review of promising progress
-
Gregorevic P, Chamberlain JS. Gene therapy for muscular dystrophy-a review of promising progress. Expert Opin Biol Ther 2003; 3: 803-814.
-
(2003)
Expert Opin Biol Ther
, vol.3
, pp. 803-814
-
-
Gregorevic, P.1
Chamberlain, J.S.2
-
53
-
-
33845218878
-
Gene therapy progress and prospects: Duchenne muscular dystrophy
-
Foster K, Foster H, Dickson JG. Gene therapy progress and prospects: Duchenne muscular dystrophy. Gene Ther 2006; 13: 1677-1685.
-
(2006)
Gene Ther
, vol.13
, pp. 1677-1685
-
-
Foster, K.1
Foster, H.2
Dickson, J.G.3
-
54
-
-
69149106708
-
Emerging strategies for cell and gene therapy of the muscular dystrophies
-
Muir LA, Chamberlain JS. Emerging strategies for cell and gene therapy of the muscular dystrophies. Expert Rev Mol Med 2009; 11: e18.
-
(2009)
Expert Rev Mol Med
, vol.11
-
-
Muir, L.A.1
Chamberlain, J.S.2
-
55
-
-
80055064588
-
Progress in therapy for Duchenne muscular dystrophy
-
Fairclough RJ, Bareja A, Davies KE. Progress in therapy for Duchenne muscular dystrophy. Exp Physiol 2011; 96: 1101-1113.
-
(2011)
Exp Physiol
, vol.96
, pp. 1101-1113
-
-
Fairclough, R.J.1
Bareja, A.2
Davies, K.E.3
-
56
-
-
79960134129
-
Internal deletion compromises the stability of dystrophin
-
Henderson DM, Belanto JJ, Li B, Heun-Johnson H, Ervasti JM. Internal deletion compromises the stability of dystrophin. Hum Mol Genet 2011; 20: 2955-2963.
-
(2011)
Hum Mol Genet
, vol.20
, pp. 2955-2963
-
-
Henderson, D.M.1
Belanto, J.J.2
Li, B.3
Heun-Johnson, H.4
Ervasti, J.M.5
-
58
-
-
0035069182
-
Binding of dystrophin's tandem calponin homology domain to F-actin is modulated by actin's structure
-
Orlova A, Rybakova IN, Prochniewicz E, Thomas DD, Ervasti JM, Egelman EH. Binding of dystrophin's tandem calponin homology domain to F-actin is modulated by actin's structure. Biophys J 2001; 80: 1926.
-
(2001)
Biophys J
, vol.80
, pp. 1926
-
-
Orlova, A.1
Rybakova, I.N.2
Prochniewicz, E.3
Thomas, D.D.4
Ervasti, J.M.5
Egelman, E.H.6
-
60
-
-
0041620499
-
Do the utrophin tandem calponin homology domains bind F-actin in a compact or extended conformation?
-
Galkin VE, Orlova A, VanLoock MS, Egelman EH. Do the utrophin tandem calponin homology domains bind F-actin in a compact or extended conformation? J Mol Biol 2003; 331: 967-972.
-
(2003)
J Mol Biol
, vol.331
, pp. 967-972
-
-
Galkin, V.E.1
Orlova, A.2
VanLoock, M.S.3
Egelman, E.H.4
-
62
-
-
77951979359
-
Opening of tandem calponin homology domains regulated their affinity for F-actin
-
Galkin VE, Orlova A, Salmazo A, Djinovic-Carugo K, Egelman EH. Opening of tandem calponin homology domains regulated their affinity for F-actin. Nat Struct Mol Biol 2010; 17: 614-616.
-
(2010)
Nat Struct Mol Biol
, vol.17
, pp. 614-616
-
-
Galkin, V.E.1
Orlova, A.2
Salmazo, A.3
Djinovic-Carugo, K.4
Egelman, E.H.5
-
63
-
-
79961213117
-
Large-scale opening of utrophin's tandem calponin homology (CH) domains upon actin binding by an induced-fit mechanism
-
Lin AY, Prochniewicz E, James ZM, Svensson B, Thomas DD. Large-scale opening of utrophin's tandem calponin homology (CH) domains upon actin binding by an induced-fit mechanism. Proc Natl Acad Sci USA 2011; 108: 12729-12733.
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, pp. 12729-12733
-
-
Lin, A.Y.1
Prochniewicz, E.2
James, Z.M.3
Svensson, B.4
Thomas, D.D.5
-
64
-
-
0038554286
-
An atomic model for actin binding by the CH domains and spectrin-repeat modules of utrophin and dystrophin
-
Sutherland-Smith AJ, Moores CA, Norwood FLM, Hatch V, Craig R, Kendrick-Jones J, Lehman W. An atomic model for actin binding by the CH domains and spectrin-repeat modules of utrophin and dystrophin. J Mol Biol 2003; 329: 15-33.
-
(2003)
J Mol Biol
, vol.329
, pp. 15-33
-
-
Sutherland-Smith, A.J.1
Moores, C.A.2
Norwood, F.L.M.3
Hatch, V.4
Craig, R.5
Kendrick-Jones, J.6
Lehman, W.7
-
65
-
-
34548847733
-
Using circular dichroism spectra to estimate protein secondary structure
-
Greenfield NJ. Using circular dichroism spectra to estimate protein secondary structure. Nat Protocols 2006; 6: 2876-2890.
-
(2006)
Nat Protocols
, vol.6
, pp. 2876-2890
-
-
Greenfield, N.J.1
-
66
-
-
0029400480
-
NMRPipe: a multidimensional spectral processing system based on UNIX pipes
-
Delaglio F, Grzesiek S, Vuister GW, Zhu G, Pfeifer J, Bax A. NMRPipe: a multidimensional spectral processing system based on UNIX pipes. J Biomol NMR 1995; 6: 277-293.
-
(1995)
J Biomol NMR
, vol.6
, pp. 277-293
-
-
Delaglio, F.1
Grzesiek, S.2
Vuister, G.W.3
Zhu, G.4
Pfeifer, J.5
Bax, A.6
-
67
-
-
0023697408
-
Unfolding free energy changes determined by the linear extrapolation method. 1. Unfolding of phenylmethanesulfonyl alpha-chymotrypsin using different denaturants
-
Santoro MM, Bolen DW. Unfolding free energy changes determined by the linear extrapolation method. 1. Unfolding of phenylmethanesulfonyl alpha-chymotrypsin using different denaturants. Biochemistry 1988; 27: 8063-8068.
-
(1988)
Biochemistry
, vol.27
, pp. 8063-8068
-
-
Santoro, M.M.1
Bolen, D.W.2
-
68
-
-
0026754044
-
A test of the linear extrapolation of unfolding free energy changes over an extended denaturant concentration range
-
Santoro MM, Bolen DW. A test of the linear extrapolation of unfolding free energy changes over an extended denaturant concentration range. Biochemistry 1992; 31: 4901-4907.
-
(1992)
Biochemistry
, vol.31
, pp. 4901-4907
-
-
Santoro, M.M.1
Bolen, D.W.2
-
69
-
-
0042121237
-
Multiple sequence alignment with the Clustal series of programs
-
Chenna R, Sugawara H, Koike T, Lopez R, Gibson TJ, Higgins DG, Thompson JD. Multiple sequence alignment with the Clustal series of programs. Nucl Acids Res 2003; 31: 3497-3500.
-
(2003)
Nucl Acids Res
, vol.31
, pp. 3497-3500
-
-
Chenna, R.1
Sugawara, H.2
Koike, T.3
Lopez, R.4
Gibson, T.J.5
Higgins, D.G.6
Thompson, J.D.7
-
73
-
-
0028820703
-
Denaturant m values and heat capacity changes: relation to changes in accessible surface areas of protein unfolding
-
Myers JK, Pace CN, Scholtz JM. Denaturant m values and heat capacity changes: relation to changes in accessible surface areas of protein unfolding. Protein Sci 1995; 4: 2138-2148.
-
(1995)
Protein Sci
, vol.4
, pp. 2138-2148
-
-
Myers, J.K.1
Pace, C.N.2
Scholtz, J.M.3
-
75
-
-
67349085538
-
Differential stabilities of alternative exon-skipped rod motifs of dystrophin
-
Ruszczak C, Mirza A, Menhart N. Differential stabilities of alternative exon-skipped rod motifs of dystrophin. Biochim Biophys Acta 2009; 1794: 921-928.
-
(2009)
Biochim Biophys Acta
, vol.1794
, pp. 921-928
-
-
Ruszczak, C.1
Mirza, A.2
Menhart, N.3
-
76
-
-
65849498675
-
A two-amino acid mutation encountered in Duchenne muscular dystrophy decreases stability of the rod domain 23 (R23) spectrin-like repeat of dystrophin
-
Legardinier S, Legrand B, Raguénès-Nicol C, Bondon A, Hardy S, Tascon C, Rumeur EL, Hubert J-F. A two-amino acid mutation encountered in Duchenne muscular dystrophy decreases stability of the rod domain 23 (R23) spectrin-like repeat of dystrophin. J Biol Chem 2009; 284: 8822-8832.
-
(2009)
J Biol Chem
, vol.284
, pp. 8822-8832
-
-
Legardinier, S.1
Legrand, B.2
Raguénès-Nicol, C.3
Bondon, A.4
Hardy, S.5
Tascon, C.6
Rumeur, E.L.7
Hubert, J.-F.8
-
77
-
-
33847306593
-
A unified mechanism for protein folding: predetermined pathways with optional errors
-
Krishna MMG, Englander SW. A unified mechanism for protein folding: predetermined pathways with optional errors. Protein Sci 2007; 16: 449-464.
-
(2007)
Protein Sci
, vol.16
, pp. 449-464
-
-
Krishna, M.M.G.1
Englander, S.W.2
-
78
-
-
77955095972
-
A biophysical map of the dystrophin rod
-
Mirza A, Sagathevan M, Sahni N, Choi L, Menhart N. A biophysical map of the dystrophin rod. Biochim Biophys Acta 2010; 1804: 1796-1809.
-
(2010)
Biochim Biophys Acta
, vol.1804
, pp. 1796-1809
-
-
Mirza, A.1
Sagathevan, M.2
Sahni, N.3
Choi, L.4
Menhart, N.5
-
79
-
-
6344285316
-
Probing the high energy states in proteins by proteolysis
-
Park C, Marqusee S. Probing the high energy states in proteins by proteolysis. J Mol Biol 2004; 343: 1467-1476.
-
(2004)
J Mol Biol
, vol.343
, pp. 1467-1476
-
-
Park, C.1
Marqusee, S.2
-
81
-
-
79960005237
-
Prediction of amyloid aggregation in vivo
-
Belli M, Ramazzotti M, Chiti F. Prediction of amyloid aggregation in vivo. EMBO Reports 2011; 12: 657-663.
-
(2011)
EMBO Reports
, vol.12
, pp. 657-663
-
-
Belli, M.1
Ramazzotti, M.2
Chiti, F.3
-
82
-
-
0027502784
-
Thioflavine T interaction with synthetic Alzheimer's disease {beta}-amyloid peptides: Detection of amyloid aggregation in solution
-
Levine-III H. Thioflavine T interaction with synthetic Alzheimer's disease {beta}-amyloid peptides: Detection of amyloid aggregation in solution. Protein Sci 1993; 2: 404-410.
-
(1993)
Protein Sci
, vol.2
, pp. 404-410
-
-
Levine III, H.1
-
83
-
-
0024352110
-
Quantitative evaluation of congo red binding to amyloid-like proteins with a beta-pleatedsheet conformation
-
Klunk WE, Pettegrew JW, Abraham DJ. Quantitative evaluation of congo red binding to amyloid-like proteins with a beta-pleatedsheet conformation. J Histochem Cytochem 1989; 37: 1273-1281.
-
(1989)
J Histochem Cytochem
, vol.37
, pp. 1273-1281
-
-
Klunk, W.E.1
Pettegrew, J.W.2
Abraham, D.J.3
-
84
-
-
0034702025
-
Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure
-
Rafael JA, Townsend ER, Squire SE, Potter AC, Chamberlain JS, Davies KE. Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure. Hum Mol Genet 2000; 9: 1357-1367.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 1357-1367
-
-
Rafael, J.A.1
Townsend, E.R.2
Squire, S.E.3
Potter, A.C.4
Chamberlain, J.S.5
Davies, K.E.6
-
85
-
-
0028906307
-
Mdx mice show progressive weakness and muscle deterioration with age
-
Pastoret C, Sebille A. Mdx mice show progressive weakness and muscle deterioration with age. J Neurol Sci 1995; 129: 97-105.
-
(1995)
J Neurol Sci
, vol.129
, pp. 97-105
-
-
Pastoret, C.1
Sebille, A.2
-
86
-
-
0022638233
-
Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse
-
Tanabe Y, Esaki K, Nomura T. Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse. Acta Neuropathol 1986; 69: 91-95.
-
(1986)
Acta Neuropathol
, vol.69
, pp. 91-95
-
-
Tanabe, Y.1
Esaki, K.2
Nomura, T.3
-
87
-
-
0027461720
-
Localization and quantitation of the chromosome 6-encoded dystrophin-related protein in normal and pathological human muscle
-
Karpati G, Carpenter S, Morris GE, Davies KE, Guerin C, Holland P. Localization and quantitation of the chromosome 6-encoded dystrophin-related protein in normal and pathological human muscle. J Neuropathol Exp Neurol 1993; 52: 119-128.
-
(1993)
J Neuropathol Exp Neurol
, vol.52
, pp. 119-128
-
-
Karpati, G.1
Carpenter, S.2
Morris, G.E.3
Davies, K.E.4
Guerin, C.5
Holland, P.6
-
88
-
-
0030848969
-
Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy
-
Deconinck AE, Rafael JA, Skinner JA, Brown SC, Potter AC, Metzinger L, Watt DJ, Dickson JG, Tinsley JM, Davies KE. Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell 1997; 90: 717-727.
-
(1997)
Cell
, vol.90
, pp. 717-727
-
-
Deconinck, A.E.1
Rafael, J.A.2
Skinner, J.A.3
Brown, S.C.4
Potter, A.C.5
Metzinger, L.6
Watt, D.J.7
Dickson, J.G.8
Tinsley, J.M.9
Davies, K.E.10
-
89
-
-
0030848338
-
Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy
-
Grady RM, Teng HB, Nichol MC, Cunningham JC, Wilkinson RS, Sanes JR. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 1997; 90: 729-738.
-
(1997)
Cell
, vol.90
, pp. 729-738
-
-
Grady, R.M.1
Teng, H.B.2
Nichol, M.C.3
Cunningham, J.C.4
Wilkinson, R.S.5
Sanes, J.R.6
-
90
-
-
33846308923
-
Viral-mediated gene therapy for the muscular dystrophies: successes, limitations, and recent advances
-
Odom GL, Gregorevic P, Chamberlain JS. Viral-mediated gene therapy for the muscular dystrophies: successes, limitations, and recent advances. Biochim Biophys Acta 2007; 1772: 243-262.
-
(2007)
Biochim Biophys Acta
, vol.1772
, pp. 243-262
-
-
Odom, G.L.1
Gregorevic, P.2
Chamberlain, J.S.3
-
91
-
-
0034622814
-
Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice
-
Ebihara S, Guibinga G-H, Gilbert R, Nalbantoglu J, Massie B, Karpati G, Petrof BJ. Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice. Physiol Genomics 2000; 3: 133-144.
-
(2000)
Physiol Genomics
, vol.3
, pp. 133-144
-
-
Ebihara, S.1
Guibinga, G.-H.2
Gilbert, R.3
Nalbantoglu, J.4
Massie, B.5
Karpati, G.6
Petrof, B.J.7
-
92
-
-
0024554228
-
The structural stability of a protein is an important determinant of its proteolytic susceptibility in Escherichia coli
-
Parsell DA, Sauer RT. The structural stability of a protein is an important determinant of its proteolytic susceptibility in Escherichia coli. J Biol Chem 1989; 264: 7590-7595.
-
(1989)
J Biol Chem
, vol.264
, pp. 7590-7595
-
-
Parsell, D.A.1
Sauer, R.T.2
-
93
-
-
0031791519
-
Proteolysis as a measure of the free energy difference between cytochrome c and its derivatives
-
Wang L, Kallenbach NR. Proteolysis as a measure of the free energy difference between cytochrome c and its derivatives. Protein Sci 1998; 7: 2460-2464.
-
(1998)
Protein Sci
, vol.7
, pp. 2460-2464
-
-
Wang, L.1
Kallenbach, N.R.2
-
94
-
-
68849124510
-
Determining protein stability in cell lysates by pulse proteolysis and Western blotting
-
Kim M-S, Song J, Park C. Determining protein stability in cell lysates by pulse proteolysis and Western blotting. Protein Sci 2009; 18: 1051-1059.
-
(2009)
Protein Sci
, vol.18
, pp. 1051-1059
-
-
Kim, M.-S.1
Song, J.2
Park, C.3
-
95
-
-
77957970501
-
The proteasome antechamber maintains substrates in the unfolded state
-
Ruschak AM, Religa TL, Breuer S, Witt S, Kay LE. The proteasome antechamber maintains substrates in the unfolded state. Nature 2010; 467: 868-871.
-
(2010)
Nature
, vol.467
, pp. 868-871
-
-
Ruschak, A.M.1
Religa, T.L.2
Breuer, S.3
Witt, S.4
Kay, L.E.5
-
96
-
-
0026760378
-
In vitro digestion of dystrophin by calcium-dependent proteases, calpains I and II
-
Cotton P, Poussard S, Mornet D, Brustis JJ, Mohammadpour M, Leger J, Ducastaing A. In vitro digestion of dystrophin by calcium-dependent proteases, calpains I and II. Biochimie 1992; 74: 565-570.
-
(1992)
Biochimie
, vol.74
, pp. 565-570
-
-
Cotton, P.1
Poussard, S.2
Mornet, D.3
Brustis, J.J.4
Mohammadpour, M.5
Leger, J.6
Ducastaing, A.7
-
97
-
-
33744949000
-
Utrophin is a calpain substrate in muscle cells
-
Courdier-Fruh I, Briguet A. Utrophin is a calpain substrate in muscle cells. Muscle Nerve 2006; 33: 753-759.
-
(2006)
Muscle Nerve
, vol.33
, pp. 753-759
-
-
Courdier-Fruh, I.1
Briguet, A.2
-
98
-
-
0033017374
-
Enteroviral protease 2A cleaves dystrophin: evidence of cytoskeletal disruption in an acquired cardiomyopathy
-
Badorff C, Lee G-H, Lamphear BJ, Martone ME, Campbell KP, Rhoads RE, Knowlton KU. Enteroviral protease 2A cleaves dystrophin: evidence of cytoskeletal disruption in an acquired cardiomyopathy. Nat Med 1999; 5: 320-326.
-
(1999)
Nat Med
, vol.5
, pp. 320-326
-
-
Badorff, C.1
Lee, G.-H.2
Lamphear, B.J.3
Martone, M.E.4
Campbell, K.P.5
Rhoads, R.E.6
Knowlton, K.U.7
-
99
-
-
0141567459
-
Physical stability of proteins in aqueous solution: mechanism and driving forces in nonnative protein aggregation
-
Chi EY, Krishnan S, Randolph TW, Carpenter JF. Physical stability of proteins in aqueous solution: mechanism and driving forces in nonnative protein aggregation. Pharmaceut Res 2003; 20: 1325-1336.
-
(2003)
Pharmaceut Res
, vol.20
, pp. 1325-1336
-
-
Chi, E.Y.1
Krishnan, S.2
Randolph, T.W.3
Carpenter, J.F.4
-
100
-
-
34547687667
-
Correlation of levels of folded recombinant p53 in Escherichia coli with thermodynamic stability in vitro
-
Mayer S, Rüdiger S, Ang HC, Joerger AC, Fersht AR. Correlation of levels of folded recombinant p53 in Escherichia coli with thermodynamic stability in vitro. J Mol Biol 2007; 372: 268-276.
-
(2007)
J Mol Biol
, vol.372
, pp. 268-276
-
-
Mayer, S.1
Rüdiger, S.2
Ang, H.C.3
Joerger, A.C.4
Fersht, A.R.5
-
101
-
-
42649110014
-
The in vivo and in vitro aggregation properties of globular proteins correlate with their conformational stability: the SH3 case
-
Espargaró A, Castillo V, de Groot NS, Ventura S. The in vivo and in vitro aggregation properties of globular proteins correlate with their conformational stability: the SH3 case. J Mol Biol 2008; 378: 1116-1131.
-
(2008)
J Mol Biol
, vol.378
, pp. 1116-1131
-
-
Espargaró, A.1
Castillo, V.2
de Groot, N.S.3
Ventura, S.4
-
102
-
-
0027204169
-
A missense mutation in the dystrophin gene in a Duchenne muscular dystrophy patient
-
Prior TW, Papp AC, Snyder PJ, Burghes AHM, Bartolo C, Sedra MS, Western LM, Mendell JR. A missense mutation in the dystrophin gene in a Duchenne muscular dystrophy patient. Nat Genet 1993; 4: 357-360.
-
(1993)
Nat Genet
, vol.4
, pp. 357-360
-
-
Prior, T.W.1
Papp, A.C.2
Snyder, P.J.3
Burghes, A.H.M.4
Bartolo, C.5
Sedra, M.S.6
Western, L.M.7
Mendell, J.R.8
-
103
-
-
20944446411
-
DNA sequence analysis for structure/function and mutation studies in Becker muscular dystrophy
-
Hamed SA, Sutherland-Smith AJ, Gorospe JRM, Kendrick-Jones J, Hoffman EP. DNA sequence analysis for structure/function and mutation studies in Becker muscular dystrophy. Clin Genet 2005; 68: 69-79.
-
(2005)
Clin Genet
, vol.68
, pp. 69-79
-
-
Hamed, S.A.1
Sutherland-Smith, A.J.2
Gorospe, J.R.M.3
Kendrick-Jones, J.4
Hoffman, E.P.5
-
104
-
-
0034805152
-
Non-toxic ubiquitous over-expression of utrophin in the mdx mouse
-
Fisher R, Tinsley JM, Phelps SR, Squire SE, Townsend ER, Martin JE, Davies KE. Non-toxic ubiquitous over-expression of utrophin in the mdx mouse. Neuromusc Disorder 2001; 11: 713-721.
-
(2001)
Neuromusc Disorder
, vol.11
, pp. 713-721
-
-
Fisher, R.1
Tinsley, J.M.2
Phelps, S.R.3
Squire, S.E.4
Townsend, E.R.5
Martin, J.E.6
Davies, K.E.7
-
105
-
-
0027424701
-
The utrophin and dystrophin genes share similarities in genomic structure
-
Pearce M, Blake DJ, Tinsley JM, Byth BC, Campbell L, Monaco AP, Davies KE. The utrophin and dystrophin genes share similarities in genomic structure. Hum Mol Genet 1993; 2: 1765-1772.
-
(1993)
Hum Mol Genet
, vol.2
, pp. 1765-1772
-
-
Pearce, M.1
Blake, D.J.2
Tinsley, J.M.3
Byth, B.C.4
Campbell, L.5
Monaco, A.P.6
Davies, K.E.7
-
106
-
-
0030010809
-
Immunohistochemical study of utrophin and dystrophin at the motor end-plate in myasthenia gravis
-
Ito H, Yoshimura T, Satoh A, Takino H, Tsujihata M, Nagataki S. Immunohistochemical study of utrophin and dystrophin at the motor end-plate in myasthenia gravis. Acta Neuropathol 1996; 92: 14-18.
-
(1996)
Acta Neuropathol
, vol.92
, pp. 14-18
-
-
Ito, H.1
Yoshimura, T.2
Satoh, A.3
Takino, H.4
Tsujihata, M.5
Nagataki, S.6
-
107
-
-
0033817038
-
Immature end-plates and utrophin deficiency in congenital myasthemic syndrome caused by ε-AChR subunit truncating mutations
-
Sieb JP, Kraner S, Rauch M, Steinlein OK. Immature end-plates and utrophin deficiency in congenital myasthemic syndrome caused by ε-AChR subunit truncating mutations. Hum Genet 2000; 107: 160-164.
-
(2000)
Hum Genet
, vol.107
, pp. 160-164
-
-
Sieb, J.P.1
Kraner, S.2
Rauch, M.3
Steinlein, O.K.4
-
108
-
-
0034970550
-
Putative tumor suppressor loci at 6q22 and 6q23-q24 are involved in the malignant progression of sporadic endocrine pancreatic tumors
-
Barghorn A, Speel EJM, Farspour B, Saremaslani P, Schmid S, Perren A, Roth J, Heitz PU, Komminoth P. Putative tumor suppressor loci at 6q22 and 6q23-q24 are involved in the malignant progression of sporadic endocrine pancreatic tumors. Am J Pathol 2001; 158: 1903-1911.
-
(2001)
Am J Pathol
, vol.158
, pp. 1903-1911
-
-
Barghorn, A.1
Speel, E.J.M.2
Farspour, B.3
Saremaslani, P.4
Schmid, S.5
Perren, A.6
Roth, J.7
Heitz, P.U.8
Komminoth, P.9
-
109
-
-
0037173037
-
Deciphering peripheral nerve myelination by using Schwann cell expression profiling
-
Nagarajan R, Le N, Mahoney H, Araki T, Milbrandt J. Deciphering peripheral nerve myelination by using Schwann cell expression profiling. Proc Natl Acad Sci USA 2002; 99: 8998-9003.
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 8998-9003
-
-
Nagarajan, R.1
Le, N.2
Mahoney, H.3
Araki, T.4
Milbrandt, J.5
-
110
-
-
0036460279
-
Increased vulnerability to kainate-induced seizures in utrophin-knockout mice
-
Knuesel I, Riban V, Zuellig RA, Schaub MC, Grady RM, Sanes JR, Fritschy J-M. Increased vulnerability to kainate-induced seizures in utrophin-knockout mice. Eur J Neurosci 2002; 15: 1474-1484.
-
(2002)
Eur J Neurosci
, vol.15
, pp. 1474-1484
-
-
Knuesel, I.1
Riban, V.2
Zuellig, R.A.3
Schaub, M.C.4
Grady, R.M.5
Sanes, J.R.6
Fritschy, J.-M.7
-
111
-
-
34548701788
-
UTRN on chromosome 6q24 is mutated in multiple tumors
-
Li Y, Huang J, Zhao Y-L, He J, Wang W, Davies KE, Nosé V, Xiao S. UTRN on chromosome 6q24 is mutated in multiple tumors. Oncogene 2007; 26: 6220-6228.
-
(2007)
Oncogene
, vol.26
, pp. 6220-6228
-
-
Li, Y.1
Huang, J.2
Zhao, Y.-L.3
He, J.4
Wang, W.5
Davies, K.E.6
Nosé, V.7
Xiao, S.8
-
112
-
-
84862808113
-
Protein stability, folding, and aggregation of the N-terminal actin binding domains of dystrophin and utrophin
-
Mallela K, Singh S, Molas J, Cabello-Villegas J, Kongari N. Protein stability, folding, and aggregation of the N-terminal actin binding domains of dystrophin and utrophin. Protein Sci 2010; 19(S1): 180.
-
(2010)
Protein Sci
, vol.19
, Issue.S1
, pp. 180
-
-
Mallela, K.1
Singh, S.2
Molas, J.3
Cabello-Villegas, J.4
Kongari, N.5
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