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Degeneration of cocultures of spinal muscular atrophy muscle cells and rat spinal cord explants is not due to secreted factors and cannot be prevented by neurotrophins
Author(s) -
Braun Serge,
Croizat Bernard,
Lagrange MarieClaude,
Poindron Philippe,
Warter JeanMarie
Publication year - 1997
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199708)20:8<953::aid-mus4>3.0.co;2-5
Subject(s) - sma* , spinal muscular atrophy , neurotrophic factors , neurotrophin , spinal cord , muscle atrophy , degeneration (medical) , atrophy , nerve growth factor , neuroscience , biology , medicine , anatomy , pathology , disease , receptor , mathematics , combinatorics
We have shown recently that cocultures of muscle cells from infantile spinal muscular atrophy (SMA) patients innervated by motoneurons of normal rat spinal cord explants undergo a degeneration process, suggesting that muscle may play a role in this atrophy, which previously has been considered to be a pure motoneuron disease. Conditional media of SMA cocultures did not affect control healthy nerve muscle cocultures. Conversely, conditioned media of control cocultures were unable to prevent degeneration of SMA cocultures. Moreover, neurotrophic factors, thought to be of help in motoneuron disease treatment, did not protect SMA cocultures from premature death. Our results suggest that the abnormal phenotype observed in nerve‐muscle coculture (1) is not due to the release of a toxic factor nor to the lack of a secreted survival factor; and (2) does not respond to neurotrophin treatment. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 953–960, 1997