Calpain 3 is important for muscle regeneration: evidence from patients with limb girdle muscular dystrophies

Simon Hauerslev, Marie-Louise Sveen, Morten Duno, Corrado Angelini, John Vissing, Thomas O Krag

48 Citations (Scopus)

Abstract

Limb girdle muscular dystrophy (LGMD) type 2A is caused by mutations in the CAPN3 gene and complete lack of functional calpain 3 leads to the most severe muscle wasting. Calpain 3 is suggested to be involved in maturation of contractile elements after muscle degeneration. The aim of this study was to investigate how mutations in the four functional domains of calpain 3 affect muscle regeneration.
Original languageEnglish
JournalB M C Musculoskeletal Disorders
Volume13
Pages (from-to)43-53
Number of pages11
ISSN1471-2474
DOIs
Publication statusPublished - 2012

Keywords

  • Adolescent
  • Adult
  • Apoptosis
  • Biological Markers
  • Biopsy
  • Blotting, Western
  • Calpain
  • Denmark
  • Dystrophin
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Linear Models
  • Male
  • Middle Aged
  • Muscle Proteins
  • Muscle, Skeletal
  • Muscular Dystrophies, Limb-Girdle
  • Muscular Dystrophy, Duchenne
  • Mutation
  • MyoD Protein
  • Myogenin
  • Myosin Heavy Chains
  • Phenotype
  • Proteins
  • Regeneration
  • Severity of Illness Index
  • Vimentin
  • Young Adult

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