Search Results

Overview

Uniprot ID	Q8C7H1
Protein Name	Methylmalonic aciduria type A homolog, mitochondrial
Gene Name	Mmaa
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
392	QIPLMERKVLSGALS
78	HTEGLSDKEQRFVDR

Function

GTPase, binds and hydrolyzes GTP (By similarity). Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis (By similarity). Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT) (By similarity). Plays a dual role as both a protectase and a reactivase for MMUT (By similarity). Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl) (By similarity). 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 (By similarity)

Protein Sequence

10 MTISTLLLSP 20 NRRLLTCLSR 30 VPSPWLLHSS 40 HPAPGPPGAL 50 PNCFGHHCTK 60 RVLLSDGFRR 70 TLCVQATLKD 80 HTEGLSDKEQ 90 RFVDRLYTGL 100 VKGQRACLAE 110 AITLVESTHT 120 RKRELAQVLL 130 QRVLALQREQ 140 ELRNQGKPLT 150 FRVGLSGPPG 160 AGKSTFIECF 170 GKMLTEQGHR 180 LSVLAVDPSS 190 CTSGGSLLGD 200 KTRMIELSRD 210 MNAYIRPSPT 220 SGTLGGVTRT 230 TNEAIVLCEG 240 GGYDIILIET 250 VGVGQSEFAV 260 ADMVDMFVLL 270 LPPAGGDELQ 280 GIKRGIIEMA 290 DLVVITKSDG 300 DLIVPARRIQ 310 AEYVSALKLL 320 RRRSEVWRPK 330 VIRISARSGE 340 GITEMWDTMR 350 EFQHQMLASG 360 ELAAKRQTQH 370 KVWMWNLIQE 380 NVLEHFKTHP 390 SIREQIPLME 400 RKVLSGALSP 410 GRAADLLLKA FKSRH

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005737	cytoplasm
Cellular Component	GO:0005829	cytosol
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] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.