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Related Experiment Videos

Structure and function of peripheral nerve myelin proteins

K Uyemura1, H Asou, Y Takeda

  • 1Department of Physiology, Keio University School of Medicine, Tokyo, Japan.

Progress in Brain Research
|January 1, 1995
PubMed
Summary

The study investigates myelin glycoproteins P0 and PASII (also known as PMP22), linking their gene locations to Charcot-Marie-Tooth disease. P0 facilitates cell adhesion and neurite outgrowth, with mutations causing disease.

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Peripheral myelin contains glycoproteins P0 and PASII (PMP22), absent in central myelin of mammals but present in lower vertebrates, suggesting a link to neural regeneration.
  • PMP22 shares homology with growth arrest specific protein; its gene locus on chromosome 17p12-p11.2 is implicated in Charcot-Marie-Tooth disease type 1A.
  • The P0 gene locus on chromosome 1q22-q23 is associated with Charcot-Marie-Tooth disease type 1B, with mutations found in patients.

Purpose of the Study:

  • To investigate the function and genetic basis of myelin glycoproteins P0 and PASII/PMP22.
  • To explore the role of P0 in cell adhesion and neurite outgrowth.
  • To identify the chromosomal locations of P0 and PMP22 genes and their association with neuropathies.

Main Methods:

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  • Sequence analysis and gene locus determination for P0 and PMP22.
  • Cell culture experiments to assess P0-mediated cell adhesion and neurite outgrowth.
  • Analysis of patient chromosomes for mutations in the P0 gene.

Main Results:

  • P0 mediates homophilic cell adhesion and neurite outgrowth; its glycopeptide is crucial for adhesion, with distinct sites for adhesion and outgrowth.
  • The human P0 gene is located at 1q22-q23, and mutations in its extracellular domain are linked to Charcot-Marie-Tooth disease type 1B.
  • The human PASII/PMP22 gene is located at 17p12-p11.2, a region associated with Charcot-Marie-Tooth disease type 1A.
  • An isoform of L1 glycoprotein (L1cs) found in Schwann cells may differ functionally from neuronal L1.

Conclusions:

  • P0 and PMP22 are key myelin proteins with roles in neural function and regeneration.
  • Mutations in P0 and PMP22 genes are directly linked to specific forms of Charcot-Marie-Tooth disease.
  • P0's adhesive and neurite-promoting functions are critical for peripheral nerve development and maintenance.