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PMID: 9600994 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Heterogeneity of subcellular localization and electrophoretic mobility of survival motor neuron (SMN) protein in mammalian neural cells and tissues.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 95 ·No. 11 ·1998-05-26 ·Pages 6492-7

Francis JW, Sandrock AW, Bhide PG, Vonsattel JP, Brown RH

Abstract

Spinal muscular atrophy is caused by defects in the survival motor neuron (SMN) gene. To better understand the patterns of expression of SMN in neuronal cells and tissues, we raised a polyclonal antibody (abSMN) against a synthetic oligopeptide from SMN exon 2. AbSMN immunostaining in neuroblastoma cells and mouse and human central nervous system (CNS) showed intense labeling of nuclear "gems," along with prominent nucleolar immunoreactivity in mouse and human CNS tissues. Strong cytoplasmic labeling was observed in the perikarya and proximal dendrites of human spinal motor neurons but not in their axons. Immunoblot analysis revealed a 34-kDa species in the insoluble protein fractions from human SY5Y neuroblastoma cells, embryonic mouse spinal cord cultures, and human CNS tissue. By contrast, a 38-kDa species was detected in the cytosolic fraction of SY5Y cells. We conclude that SMN protein is expressed prominently in both the cytoplasm and nucleus in multiple types of neurons in brain and spinal cord, a finding consistent with a role for SMN as a determinant of neuronal viability.

MeSH Terms
Animals Brain Neoplasms/metabolism,pathology Cell Line Central Nervous System/metabolism,pathology Cyclic AMP Response Element-Binding Protein Electrophoresis, Polyacrylamide Gel Humans Immunohistochemistry Mice Muscular Atrophy, Spinal/metabolism,pathology Nerve Tissue Proteins/analysis Neuroblastoma/metabolism,pathology RNA-Binding Proteins SMN Complex Proteins Spinal Cord/metabolism,pathology
Chemicals
Cyclic AMP Response Element-Binding Protein Nerve Tissue Proteins RNA-Binding Proteins SMN Complex Proteins
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
Francis J W
Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Building 149, 13th Street, Charlestown, MA 02129, USA.
Sandrock A W
Bhide P G
Vonsattel J P
Brown R H
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.95.11.6492
Published
1998-05-26
Pages
6492-7
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC27826
Subset
IM
Grants
NIA NIH HHS · 5PO1AG12992 · United States
NINDS NIH HHS · 5RO1NS34913 · United States
NICHD NIH HHS · N01-HD-2-3144 · United States
External Links
PubMed source DOI: 10.1073/pnas.95.11.6492 PMC Full Text View journal details
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