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Localization of survival motor neuron protein in human apoptotic-like and regenerating muscle fibers, and neuromuscular junctions.

Abstract
Mutations in the gene encoding survival motor neuron (SMN) protein are found in > 98% of patients with autosomal-recessive spinal muscular atrophy. We investigated the possible role of SMN in normal and abnormal human muscle by immunostaining biopsies of 20 patients with various neuromuscular diseases using monoclonal antibodies against SMN. SMN was strongly expressed cytoplasmically in chronic peripheral neuropathies, in about 80% of chronically denervated, very atrophic muscle fibers containing clumps of TUNEL-positive pyknotic nuclei: about 60% of those fibers also had cytoplasmic Bcl-2 and Bax immunoreactivity. In regenerating muscle fibers of various myopathies SMN co-localized with desmin, Bcl-2 and Bax; it was also present at the postsynaptic domain of normal human neuromuscular junctions. Thus, SMN may play a role in normal and pathological processes of adult human muscle fibers.
AuthorsA Broccolini, W K Engel, V Askanas
JournalNeuroreport (Neuroreport) Vol. 10 Issue 8 Pg. 1637-41 (Jun 03 1999) ISSN: 0959-4965 [Print] England
PMID10501549 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • BAX protein, human
  • Cyclic AMP Response Element-Binding Protein
  • Nerve Tissue Proteins
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • RNA-Binding Proteins
  • SMN Complex Proteins
  • bcl-2-Associated X Protein
Topics
  • Apoptosis
  • Cyclic AMP Response Element-Binding Protein
  • Fluorescent Antibody Technique, Direct
  • Humans
  • Immunohistochemistry
  • In Situ Nick-End Labeling
  • Muscle Fibers, Skeletal (metabolism, ultrastructure)
  • Muscle, Skeletal (metabolism, ultrastructure)
  • Nerve Tissue Proteins (biosynthesis, metabolism)
  • Neuromuscular Diseases (pathology)
  • Neuromuscular Junction (metabolism, ultrastructure)
  • Proto-Oncogene Proteins (biosynthesis)
  • Proto-Oncogene Proteins c-bcl-2 (biosynthesis)
  • RNA-Binding Proteins
  • Regeneration
  • SMN Complex Proteins
  • bcl-2-Associated X Protein

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