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Protein Page:
MMAA (human)
p Phosphorylation
a Acetylation
m Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
u Ubiquitination
s Sumoylation
n Neddylation
gl O-GlcNAc
ga O-GalNAc
h Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage

Overview
MMAA Probable GTPase. May function as chaperone. May be involved in the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Defects in MMAA are the cause of methylmalonic aciduria type cblA (MMAA); also known as methylmalonic aciduria type A or vitamin B12-responsive methylmalonicaciduria of cblA complementation type. MMAA is a disorder of methylmalonate and cobalamin metabolism due to defective synthesis of adenosylcobalamin. Inheritance is autosomal recessive. Belongs to the ArgK family. Note: This description may include information from UniProtKB.
Protein type: EC 3.6.-.-; Chaperone
Cellular Component: mitochondrial matrix
Molecular Function: GTP binding; nucleoside-triphosphatase activity
Biological Process: fatty acid beta-oxidation; vitamin metabolic process; cobalamin metabolic process; cobalamin biosynthetic process; short-chain fatty acid catabolic process; cellular lipid metabolic process; water-soluble vitamin metabolic process
Reference #:  Q8IVH4 (UniProtKB)
Alt. Names/Synonyms: cblA; methylmalonic aciduria (cobalamin deficiency) cblA type; methylmalonic aciduria type A; Methylmalonic aciduria type A protein, mitochondrial; MGC120010; MGC120011; MGC120012; MGC120013; MMAA
Gene Symbols: MMAA
Molecular weight: 46,538 Da
Basal Isoelectric point: 9.37  Predict pI for various phosphorylation states
Select Structure to View Below

MMAA

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 2 K140 QEQSNKGKPLAFRVG
0 2 T158-p PPGAGKStFIEYFGK
0 2 K165 tFIEYFGKMLTERGH
0 2 Y207 LSRDMNAYIRPSPTR
0 1 S352-p FQDLMLAsGELTAKR
  mouse

 
K137-a QELRNQGkPLTFRVG
T155-p PPGAGKStFIECFGk
K162-a tFIECFGkMLTEQGH
Y204 LSRDMNAYIRPSPTS
S349 FQHQMLASGELAAKR
  rat

 
K137 RELQNHGKPFTFRVG
T155 PPGAGKSTFIECFGK
K162 TFIECFGKMLTERGH
Y204-p LSRDMNAyIRPSPTS
S349 FQHRMLASGELAAKR
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