Overview
| Uniprot ID | Q8IVH4 |
|---|
| Protein Name | Methylmalonic aciduria type A protein, mitochondrial |
|---|
| Gene Name | MMAA |
|---|
| Organism | Homo sapiens |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 81 |
HTEGLSDKEQRFVDK |
Function
GTPase, binds and hydrolyzes GTP (PubMed:20876572, PubMed:21138732, PubMed:28497574, PubMed:28943303). Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis (PubMed:20876572, PubMed:28497574). Functions as a G protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT) (PubMed:20876572, PubMed:28497574). Plays a dual role as both a protectase and a reactivase for MMUT (PubMed:21138732, PubMed:28943303). Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl) (PubMed:21138732, PubMed:28943303). Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP (PubMed:21138732, PubMed:28943303)
Protein Sequence
10
MPMLLPHPHQ
20
HFLKGLLRAP
30
FRCYHFIFHS
40
STHLGSGIPC
50
AQPFNSLGLH
60
CTKWMLLSDG
70
LKRKLCVQTT
80
LKDHTEGLSD
90
KEQRFVDKLY
100
TGLIQGQRAC
110
LAEAITLVES
120
THSRKKELAQ
130
VLLQKVLLYH
140
REQEQSNKGK
150
PLAFRVGLSG
160
PPGAGKSTFI
170
EYFGKMLTER
180
GHKLSVLAVD
190
PSSCTSGGSL
200
LGDKTRMTEL
210
SRDMNAYIRP
220
SPTRGTLGGV
230
TRTTNEAILL
240
CEGAGYDIIL
250
IETVGVGQSE
260
FAVADMVDMF
270
VLLLPPAGGD
280
ELQGIKRGII
290
EMADLVAVTK
300
SDGDLIVPAR
310
RIQAEYVSAL
320
KLLRKRSQVW
330
KPKVIRISAR
340
SGEGISEMWD
350
KMKDFQDLML
360
ASGELTAKRR
370
KQQKVWMWNL
380
IQESVLEHFR
390
THPTVREQIP
400
LLEQKVLIGA
410
LSPGLAADFL
LKAFKSRD
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0005737 |
cytoplasm |
| Cellular Component |
GO:0005829 |
cytosol |
| Cellular Component |
GO:0005759 |
mitochondrial matrix |
| Cellular Component |
GO:0005739 |
mitochondrion |
| Molecular Function |
GO:0005525 |
GTP binding |
| Molecular Function |
GO:0003924 |
GTPase activity |
| Molecular Function |
GO:0042802 |
identical protein binding |
| Molecular Function |
GO:0140104 |
molecular carrier activity |
| Molecular Function |
GO:0042803 |
protein homodimerization activity |
| Biological Process |
GO:0009235 |
cobalamin metabolic process |
Reference
[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.