Elsevier

The Lancet

Volume 345, Issue 8951, 18 March 1995, Pages 694-695
The Lancet

Constitutive muscular abnormalities in culture in spinal muscular atrophy

https://doi.org/10.1016/S0140-6736(95)90869-2Get rights and content

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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