ASCC1

Chr 10AR

activating signal cointegrator 1 complex subunit 1

Also known as: ASC1p50, CGI-18, SMABF2, p50

The protein functions as a subunit of the activating signal cointegrator 1 (ASC-1) complex, which serves as a transcriptional coactivator for multiple transcription factors including AP-1, NF-kappa-B, and serum response factor, and also participates in DNA damage repair. Autosomal recessive mutations cause spinal muscular atrophy with congenital bone fractures, affecting both the neuromuscular system and skeletal development from birth. The gene shows relatively high tolerance to loss-of-function variants based on population data.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.322 OMIM phenotypes
Clinical SummaryASCC1
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
1.32LOEUF
pLI 0.000
Z-score 0.35
OE 0.92 (0.661.32)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.84Z-score
OE missense 0.84 (0.740.95)
173 obs / 206.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.92 (0.661.32)
00.351.4
Missense OE0.84 (0.740.95)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 22 / 23.9Missense obs/exp: 173 / 206.9Syn Z: -0.15

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ASCC1 · 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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