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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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MMAB
metabolism of cobalamin associated B
Chromosome 12 Β· 12q24.11
NCBI Gene: 326625Ensembl: ENSG00000139428.13HGNC: HGNC:19331UniProt: B4DHP4
76PubMed Papers
21Diseases
0Drugs
109Pathogenic Variants
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingcorrinoid adenosyltransferase activitytransferase activity, transferring alkyl or aryl (other than methyl) groupscobalamin bindingmethylmalonic aciduria, cblB typeVitamin B12-responsive methylmalonic acidemia type cblBmethylmalonic acidemiamethylmalonic aciduria cblb type
✦AI Summary

MMAB (metabolism of cobalamin associated B) encodes ATP:cobalamin adenosyltransferase, which catalyzes the conversion of cob(I)alamin to adenosylcobalamin (AdoCbl), the essential coenzyme for methylmalonyl-CoA mutase 1. This enzyme functions in the final step of vitamin B12 activation, directly delivering AdoCbl to MUT in an ATP-dependent manner 2. MMAB mutations cause cblB-type methylmalonic aciduria, an autosomal-recessive propionate metabolism disorder presenting with variable clinical severity correlating with specific pathogenic variants 23. Beyond its classical role in cobalamin metabolism, MMAB participates in cholesterol homeostasis regulation; hepatic MMAB expression is modulated by dietary cholesterol through SREBP2, and MMAB knockdown increases propionate and methylmalonic acid levels, which inhibit cholesterol biosynthesis and enhance LDL-receptor expression 4. Population studies indicate MMAB genetic variants associate with HDL-cholesterol levels and coronary heart disease risk in Han Chinese populations 5. Additionally, MMAB appears implicated in schizophrenia susceptibility through mitochondrial dysfunction pathways, with increased MMAB expression associated with elevated schizophrenia risk 6. During embryonic development, MMAB shows tissue-specific expression in organogenesis, suggesting roles beyond isolated metabolic functions 7.

Sources cited
1
MMAB converts cob(I)alamin to adenosylcobalamin, participates in final step of vitamin B12 conversion
PMID: 12514191
2
MMAB encodes ATP:cobalamin adenosyltransferase; mutations cause cblB-type methylmalonic aciduria; characterization of pathogenic variants and MMAB biochemistry
PMID: 34796408
3
MMAB mutations affect ATP:cob(I)alamin adenosyltransferase function and AdoCbl production; diagnostic methods for cblB patients
PMID: 23707710
4
MMAB regulates cholesterol homeostasis through SREBP2; knockdown increases propionate/methylmalonic acid, inhibiting HMGCR and cholesterol biosynthesis
PMID: 34750386
5
MMAB polymorphisms associate with HDL-cholesterol and coronary heart disease risk in Han Chinese population
PMID: 27716295
6
MMAB mitochondria-related gene associated with increased schizophrenia risk through multi-omics analysis
PMID: 40180044
7
MMAB shows tissue-specific expression patterns during mouse embryonic organogenesis
PMID: 23022071
Disease Associationsβ“˜21
methylmalonic aciduria, cblB typeOpen Targets
0.82Strong
Vitamin B12-responsive methylmalonic acidemia type cblBOpen Targets
0.82Strong
methylmalonic acidemiaOpen Targets
0.66Moderate
methylmalonic aciduria cblb typeOpen Targets
0.56Moderate
genetic disorderOpen Targets
0.49Moderate
neurodegenerative diseaseOpen Targets
0.48Moderate
Methylmalonic aciduriaOpen Targets
0.46Moderate
goutOpen Targets
0.42Moderate
hearing lossOpen Targets
0.41Moderate
myocardial infarctionOpen Targets
0.37Weak
cataractOpen Targets
0.37Weak
porokeratosis 3, disseminated superficial actinic typeOpen Targets
0.33Weak
Sensorineural hearing impairmentOpen Targets
0.33Weak
Age-related cataractOpen Targets
0.32Weak
age-related hearing impairmentOpen Targets
0.30Weak
prostate carcinomaOpen Targets
0.26Weak
obesityOpen Targets
0.23Weak
alcohol drinkingOpen Targets
0.21Weak
physical activityOpen Targets
0.15Weak
Abnormality of the skeletal systemOpen Targets
0.13Weak
Methylmalonic aciduria, cblB typeUniProt
Pathogenic Variants109
NM_052845.4(MMAB):c.556C>T (p.Arg186Trp)Pathogenic
Methylmalonic aciduria, cblB type|not provided|Methylmalonic acidemia|MMAB-related disorder|Inborn genetic diseases
β˜…β˜…β˜†β˜†2026β†’ Residue 186
NM_052845.4(MMAB):c.562G>A (p.Val188Met)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2026β†’ Residue 188
NM_052845.4(MMAB):c.568C>T (p.Arg190Cys)Pathogenic
Methylmalonic aciduria, cblB type|not provided
β˜…β˜…β˜†β˜†2026β†’ Residue 190
NM_052845.4(MMAB):c.572G>A (p.Arg191Gln)Pathogenic
Methylmalonic aciduria, cblB type|not provided|Inborn genetic diseases
β˜…β˜…β˜†β˜†2026β†’ Residue 191
NM_052845.4(MMAB):c.197-1G>TPathogenic
Methylmalonic aciduria, cblB type|Methylmalonic acidemia|Methylmalonic aciduria
β˜…β˜…β˜†β˜†2026
NM_052845.4(MMAB):c.454G>T (p.Glu152Ter)Pathogenic
Methylmalonic aciduria, cblB type|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025β†’ Residue 152
NM_052845.4(MMAB):c.577G>A (p.Glu193Lys)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 193
NM_052845.4(MMAB):c.625G>A (p.Val209Met)Pathogenic
not provided|Methylmalonic aciduria, cblB type|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025β†’ Residue 209
NM_052845.4(MMAB):c.563_577dup (p.Val188_Ala192dup)Pathogenic
not provided|Methylmalonic aciduria, cblB type|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025β†’ Residue 188
NM_052845.4(MMAB):c.581_582del (p.Arg194fs)Likely pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 194
NM_052845.4(MMAB):c.571C>T (p.Arg191Trp)Pathogenic
Methylmalonic aciduria, cblB type|not provided|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025β†’ Residue 191
NM_052845.4(MMAB):c.700C>T (p.Gln234Ter)Pathogenic
Methylmalonic aciduria, cblB type|not provided|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025β†’ Residue 234
NM_052845.4(MMAB):c.580A>G (p.Arg194Gly)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 194
NM_052845.4(MMAB):c.557G>A (p.Arg186Gln)Pathogenic
Methylmalonic aciduria, cblB type|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025β†’ Residue 186
NM_052845.4(MMAB):c.349-1G>CPathogenic
Methylmalonic aciduria, cblB type|not provided
β˜…β˜…β˜†β˜†2025
NM_052845.4(MMAB):c.357C>A (p.Cys119Ter)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 119
NM_052845.4(MMAB):c.291-1G>APathogenic
Methylmalonic aciduria, cblB type|not provided|Methylmalonic acidemia
β˜…β˜…β˜†β˜†2025
NM_052845.4(MMAB):c.290G>A (p.Gly97Glu)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 97
NM_052845.4(MMAB):c.583_584del (p.Arg195fs)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 195
NM_052845.4(MMAB):c.139C>T (p.Gln47Ter)Pathogenic
Methylmalonic aciduria, cblB type
β˜…β˜…β˜†β˜†2025β†’ Residue 47
View on ClinVar β†—
Related Genes
PCCBProtein interaction99%CD320Protein interaction97%LMBRD1Protein interaction97%MMADHCProtein interaction87%MMAAProtein interaction87%IYDProtein interaction84%
Tissue Expression6 tissues
Liver
100%
Heart
33%
Ovary
26%
Brain
23%
Lung
18%
Bone Marrow
11%
Gene Interaction Network
Click a node to explore
MMABPCCBCD320LMBRD1MMADHCMMAAIYD
PROTEIN STRUCTURE
Preparing viewer…
PDB7RUT Β· 1.50 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.87LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.58 [0.40–0.87]
RankingsWhere MMAB stands among ~20K protein-coding genes
  • #6,260of 20,598
    Most Researched76
  • #719of 5,498
    Most Pathogenic Variants109 Β· top quartile
  • #7,730of 17,882
    Most Constrained (LOEUF)0.87
Genes detectedMMAB
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
PMID: 20301409
1.00
2
High resolution melting analysis of the MMAB gene in cblB patients and in those with undiagnosed methylmalonic aciduria.
PMID: 23707710
Mol Genet Metab Β· 2013
0.90
3
Association of KCTD10, MVK, and MMAB polymorphisms with dyslipidemia and coronary heart disease in Han Chinese population.
PMID: 27716295
Lipids Health Dis Β· 2016
0.80
4
MMAB promotes negative feedback control of cholesterol homeostasis.
PMID: 34750386
Nat Commun Β· 2021
0.70
5
Integrative genetic analysis to decode the causal effect of air pollution on accelerated aging.
PMID: 40205593
QJM Β· 2025
0.60