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PIGP

Function

Part of the glycosylphosphatidylinositol-N-acetylglucosaminyltransferase (GPI-GnT) complex that catalyzes the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol and participates in the first step of GPI biosynthesis.

Involvement in disease

Developmental and epileptic encephalopathy 55

DEE55

A form of epileptic encephalopathy, a heterogeneous group of severe childhood onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE55 is an autosomal recessive condition.

None

The disease is caused by variants affecting the gene represented in this entry.

Pathway

Glycolipid biosynthesis; glycosylphosphatidylinositol-anchor biosynthesis.

Sequence similarities

Belongs to the PIGP family.

Tissue specificity

Ubiquitous.

Cellular localization

  • Membrane
  • Multi-pass membrane protein

Alternative names

  • Phosphatidylinositol N-acetylglucosaminyltransferase subunit P
  • Down syndrome critical region protein 5
  • Down syndrome critical region protein C
  • Phosphatidylinositol-glycan biosynthesis class P protein
  • PIG-P
  • DSCRC
  • DSCR5
  • NPD010
  • DCRC
  • PIGP

Target type

Proteins

Molecular weight

18089Da