Cyclosporine A treatment for Ullrich congenital muscular dystrophy: A cellular study of mitochondrial dysfunction and its rescue

Brain

Volumn 132, Issue 1, 2009, Pages 147-155

  Hicks, D ^a   Lampe, A K ^a   Laval, S H ^a   Allamand, V ^b,c   Jimenez Mallebrera, C ^d   Walter, M C ^e   Muntoni, F ^d   Quijano Roy, S ^f   Richard, P ^g   Straub, V ^a   Lochmuller H ^a   Bushby, K M D ^a,h  

^a NEWCASTLE UNIVERSITY   (United Kingdom)

^b INSERM   (France)

^c SORBONNE UNIVERSITÉ   (France)

^d UNIVERSITY COLLEGE LONDON   (United Kingdom)

^e LUDWIG MAXIMILIANS UNIVERSITY   (Germany)

^f RAYMOND POINCARÉ HOSPITAL   (France)

^g PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^h INSTITUTE OF HUMAN GENETICS   (United Kingdom)

Author keywords

Extracellular matrix; Membrane potential; Mitochondria; MPTP; Muscular dystrophy

Indexed keywords

COLLAGEN TYPE 1; COLLAGEN TYPE 6; CYCLOSPORIN A; DESMIN; DYE; LAMININ; TETRAMETHYL RHODAMINE METHYL ESTER; UNCLASSIFIED DRUG;

ADOLESCENT; ADULT; ARTICLE; BETHLEM MYOPATHY; CHILD; CLINICAL ARTICLE; COLLAGEN DEFECT; CONTROLLED STUDY; DEPOLARIZATION; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DUCHENNE MUSCULAR DYSTROPHY; EXTRACELLULAR MATRIX; FIBROBLAST CULTURE; HUMAN; HUMAN CELL; IMMUNOFLUORESCENCE; INFANT; LEIGH DISEASE; LIMB GIRDLE MUSCULAR DYSTROPHY; MEMBRANE CHANNEL; MEROSIN DEFICIENT CONGENITAL MUSCULAR DYSTROPHY; MITOCHONDRIAL MEMBRANE; MUSCULAR DYSTROPHY; MYOBLAST; MYOPATHY; PERMEABILITY TRANSITION PORE; PHENOTYPE; PRIORITY JOURNAL; SKIN FIBROBLAST; ULLRICH CONGENITAL MUSCULAR DYSTROPHY;

EID: 58849108906     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awn289     Document Type: Article

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