Characterization of a novel peripheral nervous system myelin protein (PMP-22/SR13)
TLDR
The name Peripheral Myelin Protein-22 (PMP-22) is proposed for the proteins and cDNA previously designated PASII, SR13, and gas3 that is apparently exclusively expressed in the peripheral nervous system where it is a major component of myelin.Abstract:
We have recently described a novel cDNA, SR13 (Welcher, A. A., U. Suter, M. De Leon, G. J. Snipes, and E. M. Shooter. 1991. Proc. Natl. Acad. Sci. USA. 88:7195-7199), that is repressed after sciatic nerve crush injury and shows homology to both the growth arrest-specific mRNA, gas3 (Manfioletti, G., M. E. Ruaro, G. Del Sal, L. Philipson, and C. Schneider, 1990. Mol. Cell Biol. 10:2924-2930), and to the myelin protein, PASII (Kitamura, K., M. Suzuki, and K. Uyemura. 1976. Biochim. Biophys. Acta. 455:806-816). In this report, we show that the 22-kD SR13 protein is expressed in the compact portion of essentially all myelinated fibers in the peripheral nervous system. Although SR13 mRNA was found in the central nervous system, no corresponding SR13 protein could be detected by either immunoblot analysis or by immunohistochemistry. Northern and immunoblot analysis of SR13 mRNA and protein expression during development of the peripheral nervous system reveal a pattern similar to other myelin proteins. Furthermore, we demonstrate by in situ mRNA hybridization on tissue sections and on individual nerve fibers that SR13 mRNA is produced predominantly by Schwann cells. We conclude that the SR13 protein is apparently exclusively expressed in the peripheral nervous system where it is a major component of myelin. Thus, we propose the name Peripheral Myelin Protein-22 (PMP-22) for the proteins and cDNA previously designated PASII, SR13, and gas3.read more
Citations
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Influence of aging on peripheral nerve function and regeneration.
TL;DR: Age-related changes are not linearly progressive with age; the capabilities for axonal regeneration and reinnervation are maintained throughout life, but tend to be delayed and less effective with aging.
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The gene for the peripheral myelin protein PMP-22 is a candidate for Charcot-Marie-Tooth disease type 1A.
Pragna Patel,Benjamin B. Roa,A A Welcher,A A Welcher,Schoener-Scott R,Barbara J. Trask,Liu Pentao,G J Snipes,Carlos A. Garcia,Uta Francke,Eric M. Shooter,Lupski,Ueli Suter +12 more
TL;DR: It is suggested that a gene dosage effect involving PMP–22 is at least partially responsible for the demyelinating neuropathy seen in CMT1A.
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Neuroactive steroids: A therapeutic approach to maintain peripheral nerve integrity during neurodegenerative events.
E. Leonelli,M. Ballabio,Antonio Consoli,Ilaria Roglio,Valerio Magnaghi,Roberto Cosimo Melcangi +5 more
TL;DR: The hypothesis that neuroactive steroids might represent a new therapeutic strategy for peripheral neuropathy is proposed.
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Connexin32 is a myelin-related protein in the PNS and CNS
Steven S. Scherer,Suzanne M. Deschênes,Yi-Tian Xu,Judith B. Grinspan,Kenneth H. Fischbeck,David L. Paul +5 more
TL;DR: Cx32 is expressed as part of the myelinating phenotype of both Schwann cells and oligodendrocytes, indicating that this gap junction protein plays in important role in the biology of myelin-forming cells.
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Charcot-Marie-Tooth type 1A duplication appears to arise from recombination at repeat sequences flanking the 1.5 Mb monomer unit.
TL;DR: A physical map of chromosome 17p11.2–p12, which contains a submicroscopic duplication in patients with Charcot–Marie–Tooth disease type 1A is constructed and it is proposed that the de novo CMT1A duplication arises from unequal crossing over due to misalignment at these C MT1A–REP repeat sequences during meiosis.
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Isolation and sequence of a cDNA encoding the major structural protein of peripheral myelin.
Greg Lemke,Richard Axel +1 more
TL;DR: The techniques of differential screening and hybrid selection are used to identify a cDNA clone encoding the Schwann cell glycoprotein P0, the major structural protein of the peripheral myelin sheath, and the sequence of this protein indicates that P0 is an integral membrane protein containing a single membrane-spanning region, a large hydrophobic extracellular domain, and a smaller basic intracellular domain.
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The amino acid sequences of the myelin-associated glycoproteins: homology to the immunoglobulin gene superfamily
James L. Salzer,WP Holmes,Colman +2 more
TL;DR: The existence in central nervous system myelin of two MAG polypeptides with populations of 67,000 and 72,000 that are designated small MAG (S-MAG) and large MAG (L-MAG), respectively are demonstrated and of considerable interest is the finding that the cytoplasmic domain of L- MAG, but not of S-MAG, contains an amino acid sequence that resembles the autophosphorylation site of the epidermal growth factor receptor.