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Protein Page:
MUT (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
MUT Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM). MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Belongs to the methylmalonyl-CoA mutase family. Note: This description may include information from UniProtKB.
Protein type: EC 5.4.99.2; Carbohydrate Metabolism - propanoate; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Isomerase
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: methylmalonyl-CoA mutase activity; metal ion binding; cobalamin binding
Biological Process: fatty acid beta-oxidation; vitamin metabolic process; cobalamin metabolic process; short-chain fatty acid catabolic process; homocysteine metabolic process; cellular lipid metabolic process; water-soluble vitamin metabolic process; post-embryonic development
Reference #:  P22033 (UniProtKB)
Alt. Names/Synonyms: MCM; methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase, mitochondrial; MUT; MUTA
Gene Symbols: MUT
Molecular weight: 83,134 Da
Basal Isoelectric point: 6.48  Predict pI for various phosphorylation states
Select Structure to View Below

MUT

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 1 K54 AKKQLKGKNPEDLIW
0 1 S68-p WHTPEGIsIKPLYSK
0 17 K89-ac PEELPGVkPFTRGPY
0 1 K89 PEELPGVKPFTRGPY
0 1 K89 PEELPGVKPFTRGPY
0 1 K121 STVEESNKFYKDNIK
0 1 Y146 DLATHRGYDSDNPRV
0 1 K212 EQGVPKEKLTGtIQN
0 1 K212 EQGVPKEKLTGtIQN
0 1 T216-p PKEKLTGtIQNDILK
0 1 K223 tIQNDILKEFMVRNT
0 1 S238-p YIFPPEPsMKIIADI
0 1 Y287-p TLADGLEySRTGLQA
0 1 K335-ac LWAHLIEkMFQPKNS
0 1 K340 IEkMFQPKNSKSLLL
0 1 K343 MFQPKNSKSLLLRAH
0 2 K343 MFQPKNSKSLLLRAH
0 1 Y364-p SLTEQDPyNNIVRTA
0 1 S481-p RRQARIDsGSEVIVG
0 1 K595 RQEFGESKEITSAIK
0 1 K595 RQEFGESKEITSAIK
0 2 K602 KEITSAIKRVHKFME
0 2 K602 KEITSAIKRVHKFME
0 11 K606 SAIKRVHKFMEREGR
  mouse

 
K52-ac AKKQLKGkNPEDLIW
S66 WHTPEGISIKPLYSR
K87-ac PEELPGVkPFTRGPY
K87-ub PEELPGVkPFTRGPY
K87-sc PEELPGVkPFTRGPY
K119-ac STVEESNkFYKDNIK
Y144-p DLATHRGyDSDNPRV
K210-ac EQGVPKEkLTGTIQN
K210-ub EQGVPKEkLTGTIQN
T214 PKEkLTGTIQNDILk
K221-ub TIQNDILkEFMVRNT
S236 YIFPPEPSMKIIADI
Y285 TIADGLEYCRTGLQA
K333 LWAHLIEKMFQPkNS
K338-sc IEKMFQPkNSkSLLL
K341-ac MFQPkNSkSLLLRAH
K341-sc MFQPkNSkSLLLRAH
Y362 SLTEQDPYNNIVRTA
S479 RRQARIDSGSEVIVG
K593-ac RQEFGESkEITSAIk
K593-sc RQEFGESkEITSAIk
K600-ac kEITSAIkRVNkFME
K600-sc kEITSAIkRVNkFME
K604-ac SAIkRVNkFMEREGR
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