GYG1

Chr 3

glycogenin 1

Also known as: GSD15, GYG

This gene encodes a member of the glycogenin family. Glycogenin is a glycosyltransferase that catalyzes the formation of a short glucose polymer from uridine diphosphate glucose in an autoglucosylation reaction. This reaction is followed by elongation and branching of the polymer, catalyzed by glycogen synthase and branching enzyme, to form glycogen. This gene is expressed in muscle and other tissues. Mutations in this gene result in glycogen storage disease XV. This gene has pseudogenes on chromosomes 1, 8 and 13 respectively. Alternatively spliced transcript variants encoding different isoforms have been identified.[provided by RefSeq, Sep 2010]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtGlycogen storage disease 15
UniProtPolyglucosan body myopathy 2

Clinical highlights

Gene-disease validity (ClinGen)
polyglucosan body myopathy type 2 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
15
Pubs (1 yr)
P/LP submissions
P/LP missense
1.41
LOEUF
GOF
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?
1.41LOEUF
pLI 0.000
Z-score 0.21
OE 0.95 (0.651.41)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.06Z-score
OE missense 0.99 (0.881.11)
185 obs / 187.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.95 (0.651.41)
00.351.4
Missense OE?0.99 (0.881.11)
00.61.4
Synonymous OE?0.90
01.21.6
LoF obs/exp: 18 / 19.0Missense obs/exp: 185 / 187.5Syn Z: 0.70

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GYG1 · 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.

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