PIGP

Chr 21

phosphatidylinositol glycan anchor biosynthesis class P

Also known as: DCRC, DCRC-S, DEE55, DSCR5, DSRC, EIEE55, PIG-P

This gene encodes an enzyme involved in the first step of glycosylphosphatidylinositol (GPI)-anchor biosynthesis. The GPI-anchor is a glycolipid found on many blood cells that serves to anchor proteins to the cell surface. The encoded protein is a component of the GPI-N-acetylglucosaminyltransferase complex that catalyzes the transfer of N-acetylglucosamine (GlcNAc) from UDP-GlcNAc to phosphatidylinositol (PI). This gene is located in the Down Syndrome critical region on chromosome 21 and is a candidate for the pathogenesis of Down syndrome. This gene has multiple pseudogenes and is a member of the phosphatidylinositol glycan anchor biosynthesis gene family. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Feb 2016]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDevelopmental and epileptic encephalopathy 55

Clinical highlights

Gene-disease validity (ClinGen)
developmental and epileptic encephalopathy, 55 · ARModerateconsider for supplementary testing
0
Active trials
3
Pubs (1 yr)
P/LP submissions
P/LP missense
1.37
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.37LOEUF
pLI 0.002
Z-score 0.88
OE 0.66 (0.341.37)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.56Z-score
OE missense 1.18 (0.991.40)
92 obs / 78.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.66 (0.341.37)
00.351.4
Missense OE?1.18 (0.991.40)
00.61.4
Synonymous OE?1.24
01.21.6
LoF obs/exp: 5 / 7.6Missense obs/exp: 92 / 78.1Syn Z: -1.03

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PIGP · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →