메뉴 건너뛰기




Volumn 20, Issue 5, 2010, Pages 346-354

166th ENMC International Workshop on Collagen type VI-related Myopathies, 22-24 May 2009, Naarden, The Netherlands

Author keywords

[No Author keywords available]

Indexed keywords

COLLAGEN TYPE 6; COLLAGEN TYPE 6 A1; COLLAGEN TYPE 6 A2; COLLAGEN TYPE 6 A3; CYCLOSPORIN A; CYCLOSPORIN A [3 N METHYL DEXTRO ALANINE 4 N ETHYLVALINE]; OMIGAPIL; PLACEBO; UNCLASSIFIED DRUG;

EID: 77952012386     PISSN: 09608966     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nmd.2010.02.012     Document Type: Article
Times cited : (37)

References (54)
  • 1
    • 73349096286 scopus 로고    scopus 로고
    • Autosomal recessive Bethlem myopathy
    • Gualandi F., Urciuolo A., Martoni E., et al. Autosomal recessive Bethlem myopathy. Neurology 2009, 73:1883-1891.
    • (2009) Neurology , vol.73 , pp. 1883-1891
    • Gualandi, F.1    Urciuolo, A.2    Martoni, E.3
  • 2
    • 70449522888 scopus 로고    scopus 로고
    • Autosomal recessive inheritance of classic Bethlem myopathy
    • Foley A.R., Hu Y., Zou Y., et al. Autosomal recessive inheritance of classic Bethlem myopathy. Neuromusc Disord 2009, 19:813-817.
    • (2009) Neuromusc Disord , vol.19 , pp. 813-817
    • Foley, A.R.1    Hu, Y.2    Zou, Y.3
  • 3
    • 0033583788 scopus 로고    scopus 로고
    • A heterozygous splice site mutation in COL6A1 leading to an in-frame deletion of the [alpha]1(VI) collagen chain in an Italian family affected by Bethlem myopathy
    • Pepe G., Giusti B., Bertini E., et al. A heterozygous splice site mutation in COL6A1 leading to an in-frame deletion of the [alpha]1(VI) collagen chain in an Italian family affected by Bethlem myopathy. Biochem Biophys Res Com 1999, 258:802-807.
    • (1999) Biochem Biophys Res Com , vol.258 , pp. 802-807
    • Pepe, G.1    Giusti, B.2    Bertini, E.3
  • 4
    • 53049098469 scopus 로고    scopus 로고
    • Therapy of collagen VI-related myopathies (Bethlem and Ullrich)
    • Merlini L., Bernardi P. Therapy of collagen VI-related myopathies (Bethlem and Ullrich). Neurotherapeutics 2008, 5:613-618.
    • (2008) Neurotherapeutics , vol.5 , pp. 613-618
    • Merlini, L.1    Bernardi, P.2
  • 5
    • 0036173516 scopus 로고    scopus 로고
    • Bethlem myopathy (Bethlem) 86th ENMC international workshop, 10-11 November 2000, Naarden, The Netherlands
    • Pepe G., de Visser M., Bertini E., et al. Bethlem myopathy (Bethlem) 86th ENMC international workshop, 10-11 November 2000, Naarden, The Netherlands. Neuromusc Disord 2002, 12:296-305.
    • (2002) Neuromusc Disord , vol.12 , pp. 296-305
    • Pepe, G.1    de Visser, M.2    Bertini, E.3
  • 6
    • 1342266974 scopus 로고    scopus 로고
    • Ullrich disease due to deficiency of collagen VI in the sarcolemma
    • Ishikawa H., Sugie K., Murayama K., et al. Ullrich disease due to deficiency of collagen VI in the sarcolemma. Neurology 2004, 62:620-623.
    • (2004) Neurology , vol.62 , pp. 620-623
    • Ishikawa, H.1    Sugie, K.2    Murayama, K.3
  • 7
    • 0017799279 scopus 로고
    • Further investigations on benign myopathy with autosomal dominant inheritance
    • Arts W., Bethlem J., Volkers W. Further investigations on benign myopathy with autosomal dominant inheritance. J Neurol 1978, 217:201-206.
    • (1978) J Neurol , vol.217 , pp. 201-206
    • Arts, W.1    Bethlem, J.2    Volkers, W.3
  • 8
    • 0017259099 scopus 로고
    • Benign myopathy with autosomal dominant inheritance: a report of three pedigrees
    • Bethlem J., Van Wijngaarden G.K. Benign myopathy with autosomal dominant inheritance: a report of three pedigrees. Brain 1976, 99:91-100.
    • (1976) Brain , vol.99 , pp. 91-100
    • Bethlem, J.1    Van Wijngaarden, G.K.2
  • 9
    • 0345196592 scopus 로고    scopus 로고
    • Bethlem myopathy: a slowly progressive congenital muscular dystrophy with contractures
    • Jobsis G.J., Boers J.M., Barth P.G., de Visser M. Bethlem myopathy: a slowly progressive congenital muscular dystrophy with contractures. Brain 1999, 122:649-655.
    • (1999) Brain , vol.122 , pp. 649-655
    • Jobsis, G.J.1    Boers, J.M.2    Barth, P.G.3    de Visser, M.4
  • 10
    • 0023908963 scopus 로고
    • Early-onset benign autosomal dominant limb-girdle myopathy with contractures (Bethlem myopathy)
    • Mohire M.D., Tandan R., Fries T.J., et al. Early-onset benign autosomal dominant limb-girdle myopathy with contractures (Bethlem myopathy). Neurology 1988, 38:573-580.
    • (1988) Neurology , vol.38 , pp. 573-580
    • Mohire, M.D.1    Tandan, R.2    Fries, T.J.3
  • 11
    • 0032934331 scopus 로고    scopus 로고
    • Respiratory muscle involvement in Bethlem myopathy
    • Haq R., Speer M., Chu M., Tandan R. Respiratory muscle involvement in Bethlem myopathy. Neurology 1999, 52:174-176.
    • (1999) Neurology , vol.52 , pp. 174-176
    • Haq, R.1    Speer, M.2    Chu, M.3    Tandan, R.4
  • 12
    • 33751008058 scopus 로고    scopus 로고
    • Cardiac and Pulmonary Investigations in Bethlem Myopathy
    • van der Kooi A.J., de Voogt W.G., Bertini E., et al. Cardiac and Pulmonary Investigations in Bethlem Myopathy. Arch Neurol 2006, 63:1617-1621.
    • (2006) Arch Neurol , vol.63 , pp. 1617-1621
    • van der Kooi, A.J.1    de Voogt, W.G.2    Bertini, E.3
  • 14
    • 77952009086 scopus 로고    scopus 로고
    • Predictive factors of severity and management of respiratory and orthopaedic complications in 16 Ullrich CMD patients
    • Quijano Roy S., Allamand V., Riahi N., et al. Predictive factors of severity and management of respiratory and orthopaedic complications in 16 Ullrich CMD patients. Neuromusc. Disord. 2007, 17:844.
    • (2007) Neuromusc. Disord. , vol.17 , pp. 844
    • Quijano Roy, S.1    Allamand, V.2    Riahi, N.3
  • 15
    • 68249154901 scopus 로고    scopus 로고
    • Natural history of Ullrich congenital muscular dystrophy
    • Nadeau A., Kinali M., Main M., et al. Natural history of Ullrich congenital muscular dystrophy. Neurology 2009, 73:25-31.
    • (2009) Neurology , vol.73 , pp. 25-31
    • Nadeau, A.1    Kinali, M.2    Main, M.3
  • 16
    • 41649083717 scopus 로고    scopus 로고
    • A refined diagnostic algorithm for Bethlem myopathy
    • Hicks D., Lampe A.K., Barresi R., et al. A refined diagnostic algorithm for Bethlem myopathy. Neurology 2008, 70:1192-1199.
    • (2008) Neurology , vol.70 , pp. 1192-1199
    • Hicks, D.1    Lampe, A.K.2    Barresi, R.3
  • 17
    • 33748551308 scopus 로고    scopus 로고
    • A comparative analysis of collagen VI production in muscle, skin and fibroblasts from 14 Ullrich congenital muscular dystrophy patients with dominant and recessive COL6A mutations
    • Jimenez-Mallebrera C., Maioli M.A., Kim J., et al. A comparative analysis of collagen VI production in muscle, skin and fibroblasts from 14 Ullrich congenital muscular dystrophy patients with dominant and recessive COL6A mutations. Neuromusc Disord 2006, 16:571-582.
    • (2006) Neuromusc Disord , vol.16 , pp. 571-582
    • Jimenez-Mallebrera, C.1    Maioli, M.A.2    Kim, J.3
  • 18
    • 20144389374 scopus 로고    scopus 로고
    • Muscle MRI in Ullrich congenital muscular dystrophy and Bethlem myopathy
    • Mercuri E., Lampe A., Allsop J., et al. Muscle MRI in Ullrich congenital muscular dystrophy and Bethlem myopathy. Neuromusc Disord 2005, 15:303-310.
    • (2005) Neuromusc Disord , vol.15 , pp. 303-310
    • Mercuri, E.1    Lampe, A.2    Allsop, J.3
  • 19
    • 13444293092 scopus 로고    scopus 로고
    • Automated genomic sequence analysis of the three collagen VI genes: applications to Ullrich congenital muscular dystrophy and Bethlem myopathy
    • Lampe A.K., Dunn D.M., Von Niederhausern A.C., et al. Automated genomic sequence analysis of the three collagen VI genes: applications to Ullrich congenital muscular dystrophy and Bethlem myopathy. J Med Genet 2005, 42(2):108-120.
    • (2005) J Med Genet , vol.42 , Issue.2 , pp. 108-120
    • Lampe, A.K.1    Dunn, D.M.2    Von Niederhausern, A.C.3
  • 20
    • 3042704340 scopus 로고    scopus 로고
    • Modulation of collagen fibrillogenesis by tenascin-X and type VI collagen
    • Minamitani T., Ikuta T., Saito Y., et al. Modulation of collagen fibrillogenesis by tenascin-X and type VI collagen. Exp Cell Res 2004, 298:305-315.
    • (2004) Exp Cell Res , vol.298 , pp. 305-315
    • Minamitani, T.1    Ikuta, T.2    Saito, Y.3
  • 21
    • 19944427087 scopus 로고    scopus 로고
    • Ullrich congenital muscular dystrophy: connective tissue abnormalities in the skin support overlap with Ehlers-Danlos syndromes
    • Kirschner J., Hausser I., Zou Y., et al. Ullrich congenital muscular dystrophy: connective tissue abnormalities in the skin support overlap with Ehlers-Danlos syndromes. Am J Med Genet A 2005, 132A:296-301.
    • (2005) Am J Med Genet A , vol.132 A , pp. 296-301
    • Kirschner, J.1    Hausser, I.2    Zou, Y.3
  • 22
    • 0034751165 scopus 로고    scopus 로고
    • Collagen VI deficiency affects the organization of fibronectin in the extracellular matrix of cultured fibroblasts
    • Sabatelli P., Bonaldo P., Lattanzi G., et al. Collagen VI deficiency affects the organization of fibronectin in the extracellular matrix of cultured fibroblasts. Matrix Biol 2001, 20:475-486.
    • (2001) Matrix Biol , vol.20 , pp. 475-486
    • Sabatelli, P.1    Bonaldo, P.2    Lattanzi, G.3
  • 23
    • 0042703174 scopus 로고    scopus 로고
    • Antisense suppression of collagen VI synthesis results in reduced expression of collagen I in normal human osteoblast-like cells
    • Harumiya S., Gibson M., Koshihara Y. Antisense suppression of collagen VI synthesis results in reduced expression of collagen I in normal human osteoblast-like cells. Biosci Biotechnol Biochem 2002, 66:2743-2747.
    • (2002) Biosci Biotechnol Biochem , vol.66 , pp. 2743-2747
    • Harumiya, S.1    Gibson, M.2    Koshihara, Y.3
  • 24
    • 61649119401 scopus 로고    scopus 로고
    • Developmental and osteoarthritic changes in Col6a1-knockout mice: biomechanics of type VI collagen in the cartilage pericellular matrix
    • Alexopoulos L., Youn I., Bonaldo P., Guilak F. Developmental and osteoarthritic changes in Col6a1-knockout mice: biomechanics of type VI collagen in the cartilage pericellular matrix. Arthritis Rheum. 2009, 60:771-779.
    • (2009) Arthritis Rheum. , vol.60 , pp. 771-779
    • Alexopoulos, L.1    Youn, I.2    Bonaldo, P.3    Guilak, F.4
  • 25
    • 58849099029 scopus 로고    scopus 로고
    • Collagen VI protects neurons against A[beta] toxicity
    • Cheng J.S., Dubal D.B., Kim D.H., et al. Collagen VI protects neurons against A[beta] toxicity. Nat Neurosci 2009, 12:119-121.
    • (2009) Nat Neurosci , vol.12 , pp. 119-121
    • Cheng, J.S.1    Dubal, D.B.2    Kim, D.H.3
  • 26
    • 33947109970 scopus 로고    scopus 로고
    • Mammalian collagen receptors
    • Leitinger B., Hohenester E. Mammalian collagen receptors. Matrix Biol 2007, 26:146-155.
    • (2007) Matrix Biol , vol.26 , pp. 146-155
    • Leitinger, B.1    Hohenester, E.2
  • 27
    • 0242552255 scopus 로고    scopus 로고
    • The NG2 proteoglycan: past insights and future prospects
    • Stallcup W. The NG2 proteoglycan: past insights and future prospects. J Neurocytol 2002, 31:423-435.
    • (2002) J Neurocytol , vol.31 , pp. 423-435
    • Stallcup, W.1
  • 28
    • 0034624050 scopus 로고    scopus 로고
    • Collagens serve as an extracellular store of bioactive interleukin 2
    • Somasundaram R., Ruehl M., Tiling N., et al. Collagens serve as an extracellular store of bioactive interleukin 2. J Biol Chem 2000, 275:38170-38175.
    • (2000) J Biol Chem , vol.275 , pp. 38170-38175
    • Somasundaram, R.1    Ruehl, M.2    Tiling, N.3
  • 29
    • 0036479318 scopus 로고    scopus 로고
    • Interstitial collagens I, III, and VI sequester and modulate the multifunctional cytokine oncostatin M
    • Somasundaram R., Ruehl M., Schaefer B., et al. Interstitial collagens I, III, and VI sequester and modulate the multifunctional cytokine oncostatin M. J Biol Chem 2002, 277:3242-3246.
    • (2002) J Biol Chem , vol.277 , pp. 3242-3246
    • Somasundaram, R.1    Ruehl, M.2    Schaefer, B.3
  • 30
    • 18544383777 scopus 로고    scopus 로고
    • The epithelial mitogen keratinocyte growth factor binds to collagens via the consensus sequence glycine-proline-hydroxyproline
    • Ruehl M., Somasundaram R., Schoenfelder I., et al. The epithelial mitogen keratinocyte growth factor binds to collagens via the consensus sequence glycine-proline-hydroxyproline. J Biol Chem 2002, 277:26872-26878.
    • (2002) J Biol Chem , vol.277 , pp. 26872-26878
    • Ruehl, M.1    Somasundaram, R.2    Schoenfelder, I.3
  • 31
    • 0029909655 scopus 로고    scopus 로고
    • Type I, II, III, IV, V, and VI collagens serve as extracellular ligands for the isoforms of platelet-derived growth factor (AA, BB, and AB)
    • Somasundaram R., Schuppan D. Type I, II, III, IV, V, and VI collagens serve as extracellular ligands for the isoforms of platelet-derived growth factor (AA, BB, and AB). J Biol Chem 1996, 271:26884-26891.
    • (1996) J Biol Chem , vol.271 , pp. 26884-26891
    • Somasundaram, R.1    Schuppan, D.2
  • 32
    • 42949160078 scopus 로고    scopus 로고
    • Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem
    • Zou Y.M.D., Zhang R.-Z.M.D., Sabatelli P.B., Chu M.-L.P., Bönnemann C.G.M.D. Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem. J Neuropathol Exp Neurol 2008, 67:144-154.
    • (2008) J Neuropathol Exp Neurol , vol.67 , pp. 144-154
    • Zou, Y.M.D.1    Zhang, R.-Z.M.D.2    Sabatelli, P.B.3    Chu, M.-L.P.4    Bönnemann, C.G.M.D.5
  • 33
    • 55149100037 scopus 로고    scopus 로고
    • An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells
    • Braghetta P., Ferrari A., Fabbro C., et al. An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells. Exp Cell Res 2008, 314:3508-3518.
    • (2008) Exp Cell Res , vol.314 , pp. 3508-3518
    • Braghetta, P.1    Ferrari, A.2    Fabbro, C.3
  • 34
    • 50649101286 scopus 로고    scopus 로고
    • Three novel collagen VI chains, {alpha}4(VI), {alpha}5(VI), and {alpha}6(VI)
    • Fitzgerald J., Rich C., Zhou F.H., Hansen U. Three novel collagen VI chains, {alpha}4(VI), {alpha}5(VI), and {alpha}6(VI). J Biol Chem 2008, 283:20170-20180.
    • (2008) J Biol Chem , vol.283 , pp. 20170-20180
    • Fitzgerald, J.1    Rich, C.2    Zhou, F.H.3    Hansen, U.4
  • 35
    • 44849095580 scopus 로고    scopus 로고
    • Three novel collagen VI chains with high homology to the {alpha}3 chain
    • Gara S.K., Grumati P., Urciuolo A., et al. Three novel collagen VI chains with high homology to the {alpha}3 chain. J Biol Chem 2008, 283:10658-10670.
    • (2008) J Biol Chem , vol.283 , pp. 10658-10670
    • Gara, S.K.1    Grumati, P.2    Urciuolo, A.3
  • 36
    • 69949143486 scopus 로고    scopus 로고
    • The knee osteoarthritis susceptibility locus DVWA on chromosome 3p24.3 is the 5′ part of the split COL6A4 gene
    • Wagener R., Gara S.K., Kobbe B., Paulsson M., Zaucke F. The knee osteoarthritis susceptibility locus DVWA on chromosome 3p24.3 is the 5′ part of the split COL6A4 gene. Matrix Biol 2009, 28:307-310.
    • (2009) Matrix Biol , vol.28 , pp. 307-310
    • Wagener, R.1    Gara, S.K.2    Kobbe, B.3    Paulsson, M.4    Zaucke, F.5
  • 37
    • 48349105759 scopus 로고    scopus 로고
    • Common variants in DVWA on chromosome 3p24.3 are associated with susceptibility to knee osteoarthritis
    • Miyamoto Y., Shi D., Nakajima M., et al. Common variants in DVWA on chromosome 3p24.3 are associated with susceptibility to knee osteoarthritis. Nat Genet 2008, 40:994-998.
    • (2008) Nat Genet , vol.40 , pp. 994-998
    • Miyamoto, Y.1    Shi, D.2    Nakajima, M.3
  • 38
    • 34548676800 scopus 로고    scopus 로고
    • Variants in a novel epidermal collagen gene (COL29A1) are associated with atopic dermatitis
    • Söderhäll C., Marenholz I., Kerscher T., et al. Variants in a novel epidermal collagen gene (COL29A1) are associated with atopic dermatitis. PLoS Biol 2007, 5:e242.
    • (2007) PLoS Biol , vol.5
    • Söderhäll, C.1    Marenholz, I.2    Kerscher, T.3
  • 39
    • 77952009028 scopus 로고    scopus 로고
    • Endoplasmic reticulum retention of COL6 chains in Ullrich congenital muscular dystrophy
    • Allamand V., Gartioux C., Lacène E., et al. Endoplasmic reticulum retention of COL6 chains in Ullrich congenital muscular dystrophy. Neuromusc Disord 2007, 17:833.
    • (2007) Neuromusc Disord , vol.17 , pp. 833
    • Allamand, V.1    Gartioux, C.2    Lacène, E.3
  • 40
    • 0031760509 scopus 로고    scopus 로고
    • Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy
    • Bonaldo P., Braghetta P., Zanetti M., et al. Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy. Hum Mol Genet 1998, 7:2135-2140.
    • (1998) Hum Mol Genet , vol.7 , pp. 2135-2140
    • Bonaldo, P.1    Braghetta, P.2    Zanetti, M.3
  • 41
    • 10744233522 scopus 로고    scopus 로고
    • Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency
    • Irwin W., Bergamin N., Sabatelli P., et al. Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency. Nat Genet 2003, 35:367-371.
    • (2003) Nat Genet , vol.35 , pp. 367-371
    • Irwin, W.1    Bergamin, N.2    Sabatelli, P.3
  • 42
    • 65549087972 scopus 로고    scopus 로고
    • Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice
    • Palma E., Tiepolo T., Angelin A., et al. Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice. Hum Mol Genet 2009, 18:2024-2031.
    • (2009) Hum Mol Genet , vol.18 , pp. 2024-2031
    • Palma, E.1    Tiepolo, T.2    Angelin, A.3
  • 43
    • 58849108906 scopus 로고    scopus 로고
    • Cyclosporine A treatment for Ullrich congenital muscular dystrophy: a cellular study of mitochondrial dysfunction and its rescue
    • Hicks D., Lampe A.K., Laval S.H., et al. Cyclosporine A treatment for Ullrich congenital muscular dystrophy: a cellular study of mitochondrial dysfunction and its rescue. Brain 2009, 132:147-155.
    • (2009) Brain , vol.132 , pp. 147-155
    • Hicks, D.1    Lampe, A.K.2    Laval, S.H.3
  • 45
    • 42449109035 scopus 로고    scopus 로고
    • Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies
    • Merlini L., Angelin A., Tiepolo T., et al. Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies. Proc Nat Acad Sci 2008, 105:5225-5229.
    • (2008) Proc Nat Acad Sci , vol.105 , pp. 5225-5229
    • Merlini, L.1    Angelin, A.2    Tiepolo, T.3
  • 46
    • 33846477445 scopus 로고    scopus 로고
    • Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins
    • Angelin A., Tiepolo T., Sabatelli P., et al. Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins. Proc Nat Acad Sci 2007, 104:991-996.
    • (2007) Proc Nat Acad Sci , vol.104 , pp. 991-996
    • Angelin, A.1    Tiepolo, T.2    Sabatelli, P.3
  • 47
    • 0032489392 scopus 로고    scopus 로고
    • Glyceraldehyde-3-phosphate dehydrogenase, the putative target of the antiapoptotic compounds CGP 3466 and R-(-)-deprenyl
    • Kragten E., Lalande I., Zimmermann K., et al. Glyceraldehyde-3-phosphate dehydrogenase, the putative target of the antiapoptotic compounds CGP 3466 and R-(-)-deprenyl. J Biol Chem 1998, 273:5821-5828.
    • (1998) J Biol Chem , vol.273 , pp. 5821-5828
    • Kragten, E.1    Lalande, I.2    Zimmermann, K.3
  • 48
    • 73349085584 scopus 로고    scopus 로고
    • Omigapil ameliorates the pathology of muscle dystrophy caused by laminin-{alpha}2 deficiency
    • Erb M., Meinen S., Barzaghi P., et al. Omigapil ameliorates the pathology of muscle dystrophy caused by laminin-{alpha}2 deficiency. J Pharmacol Exp Ther 2009, 331(3):787-795.
    • (2009) J Pharmacol Exp Ther , vol.331 , Issue.3 , pp. 787-795
    • Erb, M.1    Meinen, S.2    Barzaghi, P.3
  • 49
    • 0029683050 scopus 로고    scopus 로고
    • In vivo pharmacological effects of cyclosporin and some analogues
    • Borel J., Baumann G., Chapman I., et al. In vivo pharmacological effects of cyclosporin and some analogues. Adv Pharmacol 1996, 35:115-246.
    • (1996) Adv Pharmacol , vol.35 , pp. 115-246
    • Borel, J.1    Baumann, G.2    Chapman, I.3
  • 50
    • 65549104874 scopus 로고    scopus 로고
    • The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1 myopathic mice
    • Tiepolo T., Angelin A., Palma E., et al. The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1 myopathic mice. Br J Pharmacol 2009, 157:1045-1052.
    • (2009) Br J Pharmacol , vol.157 , pp. 1045-1052
    • Tiepolo, T.1    Angelin, A.2    Palma, E.3
  • 51
    • 41849118741 scopus 로고    scopus 로고
    • Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy
    • Millay D.P., Sargent M.A., Osinska H., et al. Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy. Nat Med 2008, 14:442-447.
    • (2008) Nat Med , vol.14 , pp. 442-447
    • Millay, D.P.1    Sargent, M.A.2    Osinska, H.3
  • 52
    • 53849097675 scopus 로고    scopus 로고
    • Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy
    • Reutenauer J., Dorchies O., Patthey-Vuadens O., Vuagniaux G., Ruegg U. Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy. Br J Pharmacol 2008, 155:574-584.
    • (2008) Br J Pharmacol , vol.155 , pp. 574-584
    • Reutenauer, J.1    Dorchies, O.2    Patthey-Vuadens, O.3    Vuagniaux, G.4    Ruegg, U.5
  • 53
    • 10244236553 scopus 로고    scopus 로고
    • The nonimmunosuppressive cyclosporin analogs NIM811 and UNIL025 display nanomolar potencies on permeability transition in brain-derived mitochondria
    • Hansson M.J., Mattiasson G., Månsson R., et al. The nonimmunosuppressive cyclosporin analogs NIM811 and UNIL025 display nanomolar potencies on permeability transition in brain-derived mitochondria. J Bioenerg Biomembr 2004, 36:407-413.
    • (2004) J Bioenerg Biomembr , vol.36 , pp. 407-413
    • Hansson, M.J.1    Mattiasson, G.2    Månsson, R.3
  • 54
    • 56649111213 scopus 로고    scopus 로고
    • 157th ENMC international workshop: patient registries for rare, inherited muscular disorders 25-27 January 2008 Naarden, The Netherlands
    • Sárközy A., Bushby K., Béroud C., Lochmüller H. 157th ENMC international workshop: patient registries for rare, inherited muscular disorders 25-27 January 2008 Naarden, The Netherlands. Neuromusc Disord 2008, 18:997-1001.
    • (2008) Neuromusc Disord , vol.18 , pp. 997-1001
    • Sárközy, A.1    Bushby, K.2    Béroud, C.3    Lochmüller, H.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.