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

Constitutive muscular abnormalities in culture in spinal muscular atrophy

S Braun  1 , B Croizat, M C Lagrange, J M Warter, P Poindron
Affiliations

Constitutive muscular abnormalities in culture in spinal muscular atrophy

S Braun et al. Lancet. .

Erratum in

  • Lancet 1995 Apr 22;345(8956):1060

Abstract

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.

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