ASB9

Chr X

ankyrin repeat and SOCS box containing 9

ASB9 encodes a substrate-recognition component of an E3 ubiquitin ligase complex that targets creatine kinases for proteasomal degradation. Biallelic mutations cause autosomal recessive developmental and epileptic encephalopathy with developmental delay, seizures, and brain atrophy typically manifesting in infancy. The gene shows significant constraint against loss-of-function variants (LOEUF 0.58), suggesting intolerance to reduced protein levels.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.58
Clinical SummaryASB9
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.70) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.58LOEUF
pLI 0.696
Z-score 2.33
OE 0.12 (0.040.58)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.99Z-score
OE missense 0.74 (0.620.89)
84 obs / 113.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.12 (0.040.58)
00.351.4
Missense OE0.74 (0.620.89)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 1 / 8.2Missense obs/exp: 84 / 113.5Syn Z: -0.29

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ASB9 · 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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