Normal innervation and differentiation of X-linked myotubular myopathy muscle cells in a nerve-muscle coculture system
OM Dorchies, J Laporte, S Wagner, C Hindelang… - Neuromuscular …, 2001 - Elsevier
To study the pathogenesis of X-linked recessive myotubular myopathy (XLMTM), we used a
nerve-muscle coculture system which allows the reconstitution of functional motor units in
vitro after coupling of human skeletal muscle cells with embryonic rat spinal cord explants.
We used three skeletal muscle cell lines derived from subjects with known mutations in the
MTM1 gene (two from embryonic tissues, associated with mutations predicted to give a
severe phenotype, and one from a neonate still alive at 3 years 6 months and exhibiting a …
nerve-muscle coculture system which allows the reconstitution of functional motor units in
vitro after coupling of human skeletal muscle cells with embryonic rat spinal cord explants.
We used three skeletal muscle cell lines derived from subjects with known mutations in the
MTM1 gene (two from embryonic tissues, associated with mutations predicted to give a
severe phenotype, and one from a neonate still alive at 3 years 6 months and exhibiting a …