CYP4V2

Chr 4AR

cytochrome P450 family 4 subfamily V member 2

Also known as: BCD, CYP4AH1

This gene encodes a member of the cytochrome P450 hemethiolate protein superfamily which are involved in oxidizing various substrates in the metabolic pathway. It is implicated in the metabolism of fatty acid precursors into n-3 polyunsaturated fatty acids. Mutations in this gene result in Bietti crystalline corneoretinal dystrophy. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Bietti crystalline corneoretinal dystrophyMIM #210370
AR

Clinical highlights

Gene-disease validity (ClinGen)
Bietti crystalline corneoretinal dystrophy · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
5
Active trials
34
Pubs (1 yr)
P/LP submissions
P/LP missense
1.03
LOEUF
LOF
Mechanism· G2P
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GeneReview available — CYP4V2
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.03LOEUF
pLI 0.000
Z-score 1.42
OE 0.70 (0.491.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.12Z-score
OE missense 1.02 (0.931.12)
305 obs / 299.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.70 (0.491.03)
00.351.4
Missense OE?1.02 (0.931.12)
00.61.4
Synonymous OE?0.89
01.21.6
LoF obs/exp: 19 / 27.0Missense obs/exp: 305 / 299.1Syn Z: 0.92

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CYP4V2 · protein map & ClinVar variants

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