CA5B

Chr X

carbonic anhydrase 5B

Also known as: CA-VB, CAVB

The protein is a mitochondrial carbonic anhydrase that catalyzes the reversible conversion of carbon dioxide to bicarbonate, participating in cellular acid-base balance and metabolic processes. Currently, no Mendelian diseases have been definitively associated with CA5B mutations in humans. The gene shows low constraint against loss-of-function variants (pLI = 0.0003, LOEUF = 1.2), suggesting tolerance to complete gene loss.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.20
Clinical SummaryCA5B
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
1.20LOEUF
pLI 0.000
Z-score 1.11
OE 0.64 (0.361.20)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.05Z-score
OE missense 1.01 (0.881.17)
125 obs / 123.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.64 (0.361.20)
00.351.4
Missense OE1.01 (0.881.17)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 7 / 11.0Missense obs/exp: 125 / 123.5Syn Z: 0.29
DN
0.7228th %ile
GOF
0.6344th %ile
LOF
0.2873th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CA5B · 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.

Search ClinicalTrials.gov →