Constitutive muscular abnormalities in culture in spinal muscular atrophy

Lancet. 1995 Mar 18;345(8951):694-5. doi: 10.1016/s0140-6736(95)90869-2.


To explore the cause of spinal muscular atrophy (SMA), we used an in-vitro model of nerve-muscle co-cultures in which motoneurons were normal and satellite cells were obtained from SMA patients. In co-cultures initiated with satellite cells from type I and type II SMA patients only, we observed degeneration of the innervated fibres after 1-3 weeks of nerve-muscle co-culture. This process involved vacuolisation, disorganisation, and death of the innervated muscle fibres. This observation points to a muscular implication in the severe forms of SMAs.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Culture Techniques
  • Humans
  • Muscle Fibers, Skeletal / pathology
  • Muscles / innervation
  • Muscles / pathology
  • Muscular Atrophy, Spinal / pathology*
  • Neuromuscular Diseases / pathology
  • Neurons / pathology