myelin P0 protein; myelin protein zero; myelin peripheral protein; MPP (MPZ)

Function

• the major glycoprotein of myelin in the peripheral nervous system
• creation of an extracellular membrane face which guides the wrapping process & ultimately compacts adjacent myelin lamellae

Structure

• N-glycosylated; contains sulfate-substituted glycan
• belongs to the myelin P0 protein family
• contains 1 Ig-like V-type domain (immunoglobulin-like)

Compartment

membrane, myelin

Expression

restricted to peripheral nervous system Schwann cells

Pathology

• mutations in the MPZ gene are seen in
  • Charcot-Marie-Tooth neuropathy type-1B
  • Dejerine-Sottas syndrome
• defects in MPZ are the cause of
  • Charcot-Marie-Tooth disease type 1B (CMT1B)
  • Charcot-Marie-Tooth disease type 2I
  • Charcot-Marie-Tooth disease type 2J (CMT2J)
  • Adie pupil
  • Charcot-Marie-Tooth disease dominant intermediate D
  • Dejerine-Sottas syndrome
  • congenital hypomyelination neuropathy
  • Roussy-Levy syndrome

More general terms

• myelin protein
• glycoprotein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4359
• OMIM: https://mirror.omim.org/entry/103100
• OMIM: https://mirror.omim.org/entry/118200
• OMIM: https://mirror.omim.org/entry/145900
• OMIM: https://mirror.omim.org/entry/159440
• OMIM: https://mirror.omim.org/entry/180800
• OMIM: https://mirror.omim.org/entry/605253
• OMIM: https://mirror.omim.org/entry/607677
• OMIM: https://mirror.omim.org/entry/607736
• OMIM: https://mirror.omim.org/entry/607791
• UniProt: http://www.uniprot.org/uniprot/P25189.html