PIGW

Chr 17

phosphatidylinositol glycan anchor biosynthesis class W

Also known as: Gwt1, HPMRS5

The protein encoded by this gene is an inositol acyltransferase that acylates the inositol ring of phosphatidylinositol. This occurs in the endoplasmic reticulum and is a step in the biosynthesis of glycosylphosphatidylinositol (GPI), which anchors many cell surface proteins to the membrane. Defects in this gene are a cause of the age-dependent epileptic encephalopathy West syndrome as well as a syndrome exhibiting hyperphosphatasia and cognitive disability (HPMRS5). [provided by RefSeq, Jul 2017]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtGlycosylphosphatidylinositol biosynthesis defect 11

Clinical highlights

Gene-disease validity (ClinGen)
congenital disorder of glycosylation · ARLimitednot for standalone diagnostic reporting
0
Active trials
5
Pubs (1 yr)
P/LP submissions
P/LP missense
1.07
LOEUF
DN
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?
1.07LOEUF
pLI 0.000
Z-score 1.35
OE 0.63 (0.391.07)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.05Z-score
OE missense 0.99 (0.901.10)
256 obs / 258.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.63 (0.391.07)
00.351.4
Missense OE?0.99 (0.901.10)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 10 / 15.8Missense obs/exp: 256 / 258.2Syn Z: -0.46

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PIGW · 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 →