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Protein Page:
OXCT1 (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
OXCT1 Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. Defects in OXCT1 are a cause of succinyl-CoA-3-ketoacid- CoA transferase deficiency (SCOTD). A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. Belongs to the 3-oxoacid CoA-transferase family. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; EC 2.8.3.5; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Transferase; Lipid Metabolism - synthesis and degradation of ketone bodies
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: protein homodimerization activity; 3-oxoacid CoA-transferase activity
Biological Process: response to drug; heart development; response to hormone stimulus; positive regulation of insulin secretion; cellular lipid metabolic process; ketone body metabolic process; response to starvation; response to ethanol; ketone body catabolic process; ketone catabolic process; brain development; response to activity; response to nutrient
Reference #:  P55809 (UniProtKB)
Alt. Names/Synonyms: 3-oxoacid CoA transferase 1; 3-oxoacid-CoA transferase 1; OXCT; OXCT1; SCOT; SCOT-s; SCOT1; somatic-type succinyl CoA:3-oxoacid CoA-transferase; Somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
Gene Symbols: OXCT1
Molecular weight: 56,158 Da
Basal Isoelectric point: 7.13  Predict pI for various phosphorylation states
Select Structure to View Below

OXCT1

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 K41 TSAHRHTKFYTDPVE
0 37 Y115-p IKRMVSSyVGENAEF
0 7 S170-p TLVQEGGsPIKYNKD
0 1 K185 GSVAIASKPREVREF
0 5 K406-a LGAMQVSkYGDLANW
0 1 K418 ANWMIPGKMVkGMGG
0 5 K421-a MIPGKMVkGMGGAMD
0 1 K421 MIPGKMVKGMGGAMD
0 2 S431-p GGAMDLVssAKTKVV
0 2 S432-p GAMDLVssAKTKVVV
0 1 K455 NAHKIMEKCTLPLTG
0 18 K480-a KAVFDVDkkkGLTLI
0 52 K481-a AVFDVDkkkGLTLIE
0 17 K482-a VFDVDkkkGLTLIEL
  mouse

 
K41-a VSTRHHTkFYTDPVE
Y115 IKRMISSYVGENAEF
S170 TLVQEGGSPIKYNKD
K185-a GSVAIASkPREVREF
K406 LGAMQVSKYGDLANW
K418-a ANWMIPGkMVkGMGG
K421-a MIPGkMVkGMGGAMD
K421-u MIPGkMVkGMGGAMD
S431 GGAMDLVSSSKTKVV
S432 GAMDLVSSSKTKVVV
K455-a NAHKIMEkCTLPLTG
K480-a KGVFDVDkKNGLTLI
K481 GVFDVDkKNGLTLIE
N482 VFDVDkKNGLTLIEL
  rat

 
K41 VSTRHHTKFYTDPVE
Y115-p IKRMISSyVGENAEF
S170 TLVQEGGSPIKYNKD
K185 GSVAIASKPREVREF
K406 LGAMQVSKYGDLANW
K418 ANWMIPGKMVKGMGG
K421 MIPGKMVKGMGGAMD
K421 MIPGKMVKGMGGAMD
S431 GGAMDLVSSSKTKVV
S432 GAMDLVSSSKTKVVV
K455 NAHKIMEKCTLPLTG
K480 KGVFDVDKKNGLTLI
K481 GVFDVDKKNGLTLIE
N482 VFDVDKKNGLTLIEL
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