MMAB

Chr 12AR

metabolism of cobalamin associated B

Also known as: ATR, CFAP23, cblB, cob

This gene encodes a protein that catalyzes the final step in the conversion of vitamin B(12) into adenosylcobalamin (AdoCbl), a vitamin B12-containing coenzyme for methylmalonyl-CoA mutase. Mutations in the gene are the cause of vitamin B12-dependent methylmalonic aciduria linked to the cblB complementation group. Alternatively spliced transcript variants have been found. [provided by RefSeq, Apr 2011]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.881 OMIM phenotype
Clinical SummaryMMAB
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Gene-Disease Validity (ClinGen)
methylmalonic aciduria, cblB type · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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?
0.88LOEUF
pLI 0.004
Z-score 1.88
OE 0.45 (0.240.88)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.11Z-score
OE missense 1.03 (0.901.17)
152 obs / 148.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.45 (0.240.88)
00.351.4
Missense OE?1.03 (0.901.17)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 6 / 13.4Missense obs/exp: 152 / 148.3Syn Z: -0.45
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveMMAB-related methylmalonic aciduriaLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7033th %ile
GOF
0.5562th %ile
LOF
0.3067th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MMAB · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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