UGP2

Chr 2AR

UDP-glucose pyrophosphorylase 2

Also known as: DEE83, EIEE83, SVUGP2, UDPG, UDPGP, UDPGP2, UGP1, UGPP1

UTP-glucose-1-phosphate uridylyltransferase catalyzes the conversion of glucose-1-phosphate to UDP-glucose, a crucial precursor for glycogen synthesis in liver and muscle. Mutations cause developmental and epileptic encephalopathy 83, inherited in an autosomal recessive pattern. The gene shows minimal constraint against loss-of-function variants (pLI near zero), consistent with the recessive inheritance pattern where heterozygous carriers are unaffected.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.121 OMIM phenotype
Clinical SummaryUGP2
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
1.12LOEUF
pLI 0.000
Z-score 1.15
OE 0.73 (0.481.12)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.93Z-score
OE missense 0.67 (0.590.76)
180 obs / 269.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.73 (0.481.12)
00.351.4
Missense OE0.67 (0.590.76)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 15 / 20.7Missense obs/exp: 180 / 269.4Syn Z: -0.60

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

UGP2 · 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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