Constitutive muscular abnormalities in culture in spinal muscular atrophy.
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.
Braun
S
S
Département d'Immunologie et d'Immunopharmacologie, Université Louis Pasteur de Strasbourg, Illkirch, France.
Croizat
B
B
Lagrange
M C
MC
Warter
J M
JM
Poindron
P
P
D046508:Culture Techniques; D006801:Humans; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D009132:Muscles / Q000294:innervation / Q000473:pathology; D009134:Muscular Atrophy, Spinal / Q000473:pathology*; D009468:Neuromuscular Diseases / Q000473:pathology; D009474:Neurons / Q000473:pathology