title: Expression of myosin isoforms and of desmin, vimentin and titin in Tunisian Duchenne-like autosomal recessive muscular dystrophy.
abstract: Morphological, morphometrical, histoenzymological, immunocytochemical and biochemical analysis were performed on muscle biopsies taken from patients suffering from tunisian autosomal recessive Duchenne-like muscular dystrophy (TDLMD) selected both by Duchenne-like clinical criteria and by the presence of normal dystrophin. Data were compared to that obtained from DMD biopsies characterized by the absence of dystrophin. The distribution of myosin heavy chain isoforms, desmin, vimentin and titin were determined in type I and type II muscle fibers. The protein pattern appeared to be less affected in TDLMD than in DMD biopsies. The regenerating fibers were mainly but not exclusively type IIC; a noticeable percentage of both type I and type II fibers coexpressed fast and slow MHC isoforms in TDLMD. This percentage was lower than in DMD. The expression of embryonic, fetal, and fast/slow myosin isoforms in type IIC fibers in TDLMD and DMD suggest different fiber type transformations in these two diseases.
authors.affiliation: Institut National de Neurologie, Laboratoire de Neuropathologie et de Neurobiologie Moléculaire, Tunisia.
authors.forename: C B
authors.initials: CB
authors.lastname: Hamida
authors.forename: N
authors.initials: N
authors.lastname: Soussi-Yanicostas
authors.forename: K
authors.initials: K
authors.lastname: Bejaoui
authors.forename: G S
authors.initials: GS
authors.lastname: Butler-Browne
authors.forename: F
authors.initials: F
authors.lastname: Hentati
authors.forename: M
authors.initials: M
authors.lastname: Ben Hamida
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001706:Biopsy; D002648:Child; D064211:Connectin; D003285:Contractile Proteins / Q000032:analysis / Q000378:metabolism; D003893:Desmin / Q000032:analysis / Q000378:metabolism*; D005260:Female; D015870:Gene Expression; D005808:Genes, Recessive; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008565:Membrane Proteins / Q000032:analysis / Q000378:metabolism; D009124:Muscle Proteins / Q000032:analysis / Q000378:metabolism*; D009132:Muscles / Q000378:metabolism* / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D009218:Myosins / Q000032:analysis / Q000378:metabolism*; D011494:Protein Kinases; D014746:Vimentin / Q000032:analysis / Q000378:metabolism*
chemical_list: D064211:Connectin; D003285:Contractile Proteins; D003893:Desmin; D008565:Membrane Proteins; D009124:Muscle Proteins; C577285:TTN protein, human; D014746:Vimentin; D011494:Protein Kinases; D009218:Myosins