UBAC1

Chr 9

UBA domain containing 1

UBAC1 encodes a non-catalytic adapter component of the KPC E3 ubiquitin ligase complex that promotes transfer of polyubiquitinated target proteins to the proteasome for degradation, regulating cell cycle progression and NF-kappa-B signaling. Mutations cause autosomal recessive neurodevelopmental disorder with seizures, spasticity, and intellectual disability. The gene shows tolerance to loss-of-function variants (pLI 0.001), consistent with the recessive inheritance pattern observed in affected patients.

OMIMResearchSummary from UniProt
LOEUF 0.74
Clinical SummaryUBAC1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.74LOEUF
pLI 0.001
Z-score 2.39
OE 0.41 (0.240.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.42Z-score
OE missense 0.93 (0.831.03)
234 obs / 252.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.41 (0.240.74)
00.351.4
Missense OE0.93 (0.831.03)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 8 / 19.4Missense obs/exp: 234 / 252.9Syn Z: 0.00

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

UBAC1 · 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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