GDPGP1

Chr 15

GDP-D-glucose phosphorylase 1

Also known as: C15orf58, VTC2

The protein functions as a GDP-D-glucose phosphorylase that regulates cellular GDP-D-glucose levels and plays a key role in neuronal stress resistance by modulating neuronal glycogen levels and maintaining glucose balance. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly, typically presenting in early infancy with seizures and severe developmental delay. The gene shows tolerance to loss-of-function variants (LOEUF 1.84), suggesting the pathogenic variants may have more complex effects on protein function.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.84
Clinical SummaryGDPGP1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.84LOEUF
pLI 0.000
Z-score -0.63
OE 1.23 (0.761.84)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.18Z-score
OE missense 0.97 (0.871.08)
221 obs / 228.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.23 (0.761.84)
00.351.4
Missense OE0.97 (0.871.08)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 11 / 9.0Missense obs/exp: 221 / 228.5Syn Z: -0.88

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GDPGP1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC