Constitutive muscular abnormalities in culture in spinal muscular atrophy
S Braun, JM Warter, P Poindron, B Croizat… - The Lancet, 1995 - Elsevier
S Braun, JM Warter, P Poindron, B Croizat, MC Lagrange
The Lancet, 1995•ElsevierTo 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 …
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 …
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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