myelin regulatory factor; myelin gene regulatory factor; contains: myelin regulatory factor, N-terminal; myelin regulatory factor, C-terminal (MYRF, C11orf9, KIAA0954, MRF)

Function

• myelin regulatory factor:
  • precursor of the transcription factor
  • mediates autocatalytic cleavage that releases the myelin regulatory factor, N-terminal component that specifically activates transcription of central nervous system (CNS) myelin genes
• myelin regulatory factor, C-terminal:
  • membrane-bound part
  • has no transcription factor activity
  • remains attached to the endoplasmic reticulum membrane following cleavage
• myelin regulatory factor, N-terminal:
  • transcription factor
  • specifically activates expression of myelin genes such as MBP, MOG, MAG & PLP1 during oligodendrocyte (OL) maturation,
  • role in oligodendrocyte maturation & CNS myelination
  • specifically recognizes & binds DNA sequence 5'-CTGGYAC-3' in the regulatory regions of myelin-specific genes & directly activates their expression
  • not only required during oligodendrocyte differentiation but is also required on an ongoing basis for the maintenance of expression of myelin genes & for the maintenance of a mature, viable oligodendrocyte phenotype

Structure

• homotrimer
• myelin regulatory factor, N-terminal:
  • the nuclear localization signals mediate translocation to the nucleus
• myelin regulatory factor:
  • the peptidase S74 domain {intramolecular chaperone auto- processed (ICA) domain or intramolecuar chaperone domain (ICD)} has protease activity & mediates autocatalytic processing of the protein to generate
    • the myelin regulatory factor,
    • N-terminal active transcription factor
    • myelin regulatory factor, C-terminal components
• belongs to the MRF family
• contains 1 NDT80 DNA-binding domain
• contains 1 peptidase S74 domain

Compartment

• myelin regulatory factor:
  • endoplasmic reticulum membrane; single-pass membrane protein
• myelin regulatory factor, N-terminal:
  • nucleus, cytoplasm
  • translocates from cytoplasm to nucleus upon autocatalytic cleavage
• myelin regulatory factor, C-terminal:
  • endoplasmic reticulum membrane; single-pass membrane protein

Alternative splicing

named isoforms=2

Expression

• expressed in lung, ARPE-19 cell line, brainstem, uterus &, to a lesser extent, in basal ganglion & liver
• weakly expressed in cerebellum & retina

More general terms

• DNA-binding protein
• glycoprotein
• membrane protein
• oligomerizing protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=745
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:745
• OMIM: https://mirror.omim.org/entry/608329
• UniProt: http://www.uniprot.org/uniprot/Q9Y2G1.html