ASPHD1

Chr 16

aspartate beta-hydroxylase domain containing 1

The ASPHD1 protein is predicted to function as a dioxygenase and localizes to cellular membranes. Mutations in this gene cause developmental and epileptic encephalopathy, with patients typically presenting with early-onset seizures, developmental delay, and intellectual disability. This gene follows an autosomal recessive inheritance pattern and shows minimal constraint against loss-of-function variants.

ResearchSummary from RefSeq
LOEUF 1.00
Clinical SummaryASPHD1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.00LOEUF
pLI 0.002
Z-score 1.58
OE 0.50 (0.281.00)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.29Z-score
OE missense 0.77 (0.680.86)
184 obs / 240.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.50 (0.281.00)
00.351.4
Missense OE0.77 (0.680.86)
00.61.4
Synonymous OE0.82
01.21.6
LoF obs/exp: 6 / 11.9Missense obs/exp: 184 / 240.5Syn Z: 1.44

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ASPHD1 · 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