ALDH1A1

Chr 9

aldehyde dehydrogenase 1 family member A1

Also known as: ALDC, ALDH-E1, ALDH1, ALDH11, HEL-9, HEL-S-53e, HEL12, PUMB1

The cytosolic aldehyde dehydrogenase enzyme catalyzes the oxidation of aldehydes to carboxylic acids, including the conversion of retinaldehyde to retinoic acid in vitamin A metabolism and detoxification of cytotoxic aldehydes from lipid peroxidation. Mutations cause microphthalmia with coloboma 8, an autosomal recessive disorder affecting ocular development. The gene is highly constrained against loss-of-function variants, indicating that complete loss of function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.34
Clinical SummaryALDH1A1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.95). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.34LOEUF
pLI 0.954
Z-score 4.13
OE 0.15 (0.070.34)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.66Z-score
OE missense 0.72 (0.640.81)
199 obs / 276.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.15 (0.070.34)
00.351.4
Missense OE0.72 (0.640.81)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 4 / 27.3Missense obs/exp: 199 / 276.4Syn Z: -0.34

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ALDH1A1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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