title: Muscle apoptosis in humans occurs in normal and denervated muscle, but not in myotonic dystrophy, dystrophinopathies or inflammatory disease.
abstract: Recent data suggest that death of muscle cells during development and in selected pathological conditions occurs via apoptosis. We investigated the occurrence of apoptosis in normal and pathological human skeletal muscle, using in situ end-labeling (ISEL) to detect DNA fragmentation, and immunohistochemistry for the expression of tissue transglutaminase and interleukin-1beta-converting enzyme (ICE)-like proteases. In normal subjects, apoptotic myonuclei were occasionally observed as evidence of normal tissue turnover. Myonuclear apoptosis due to a deficit of trophic support from nerve cells also occurred in spinal muscular atrophies. No apoptosis of muscle cells was found in dystrophinopathies, myotonic dystrophy and inflammatory myopathies, suggesting that death of myofibers in those conditions is not due to activation of a gene-directed program of death. In dystrophinopathies and inflammatory myopathies, apoptosis was found in interstitial mononuclear cells, as a likely mechanism of clearance of the inflammatory infiltrates.
authors.affiliation: Department of Neuroscience, University of Turin, Italy. palmucci@golgi.molinette.unito.it
authors.forename: A
authors.initials: A
authors.lastname: Migheli
authors.forename: T
authors.initials: T
authors.lastname: Mongini
authors.forename: C
authors.initials: C
authors.lastname: Doriguzzi
authors.forename: L
authors.initials: L
authors.lastname: Chiadò-Piat
authors.forename: R
authors.initials: R
authors.lastname: Piva
authors.forename: I
authors.initials: I
authors.lastname: Ugo
authors.forename: L
authors.initials: L
authors.lastname: Palmucci
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017209:Apoptosis; D053148:Caspase 3; D020169:Caspases / Q000032:analysis; D002467:Cell Nucleus / Q000235:genetics; D002648:Child; D053938:DNA Fragmentation; D006801:Humans; D007150:Immunohistochemistry; D020287:In Situ Nick-End Labeling; D007223:Infant; D008875:Middle Aged; D009121:Muscle Denervation; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology* / Q000648:ultrastructure; D009135:Muscular Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009220:Myositis / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009223:Myotonic Dystrophy / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011503:Transglutaminases / Q000032:analysis
chemical_list: D011503:Transglutaminases; C505407:CASP3 protein, human; D053148:Caspase 3; D020169:Caspases