title: Different pattern of HSP47 expression in skeletal muscle of patients with neuromuscular diseases.
abstract: Heat shock protein (HSP) 47, a collagen-specific molecular chaperone, is involved in the processing and secretion of procollagens, and its expression is increased in various fibrotic diseases. However, its involvement in muscle diseases is unknown. In this study, we analyzed HSP47 expression in muscular dystrophies and other muscle diseases. We found an overexpression of HSP47 in fibrous connective tissue and in the adjacent muscle membrane in various muscular dystrophies. However, in Ullrich congenital muscular dystrophy (UCMD), the overexpression of HSP47 was found only in the connective tissue, and not in the muscle membrane. The overexpression of HSP47 was found only in the muscle membrane in the case of active inflammatory myopathy. In particular, HSP47 was strongly expressed in the membrane of regenerating fibers. We found that HSP47 in the muscle membrane locates in the basement membrane with confocal microscopy. Our findings suggest that HSP47 may be involved in the repair or regeneration of muscle fibers in addition to the fibrotic change in the connective tissue.
authors.affiliation: Department of Neurology and Geriatrics, Faculty of Medicine, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8520, Japan. ihiguchi@m2.kufm.kagoshima-u.ac.jp
authors.forename: Itsuro
authors.initials: I
authors.lastname: Higuchi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hashiguchi
authors.forename: Eiji
authors.initials: E
authors.lastname: Matsuura
authors.forename: Keiko
authors.initials: K
authors.lastname: Higashi
authors.forename: Tadafumi
authors.initials: T
authors.lastname: Shiraishi
authors.forename: Noriko
authors.initials: N
authors.lastname: Hirata
authors.forename: Kimiyoshi
authors.initials: K
authors.lastname: Arimura
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Osame
mesh_terms: D001706:Biopsy / Q000379:methods; D024142:Collagen Type VI / Q000378:metabolism; D015870:Gene Expression / Q000502:physiology*; D050958:HSP47 Heat-Shock Proteins / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000473:pathology; D009468:Neuromuscular Diseases / Q000145:classification / Q000473:pathology*
chemical_list: D024142:Collagen Type VI; D050958:HSP47 Heat-Shock Proteins; C494660:SERPINH1 protein, human