catalog number :
MBS9420519
products type :
Recombinant Protein
products full name :
Recombinant Human Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
products short name :
[Succinyl-CoA:3-ketoacid coenzyme A transferase 1]
products name syn :
[3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxo; acid CoA-transferase; SCOT-s]
other names :
[succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; 3-oxoacid CoA-transferase 1; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-s]
products gene name :
[SCOT1]
other gene names :
[OXCT1; OXCT1; OXCT; SCOT; OXCT; SCOT; SCOT-s]
sequence positions :
[Full Length, 40-520aa]
sequence :
TPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAETTVVEVEEIVDIGAFAPEDIHIPQIYVHRLlKGEKYEKRIERLSIRKEGDGEAKSAKPGDDVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLlNAGKETVTILPGASFFSSDESFAMIRGGHVDL TMLGAMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPL TGKQCVNRIITEKAVFDVDKKKGL TLELWEGL TVDDVQKSTGCDFAVSPKLMPMQQIAN
TKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALL
KTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVG
ENAEFERQYLSGELEVEL
purity :
Greater than 90% as determined by SDS-PAGE.
form :
Tris-based buffer 50% glycerol.
storage stability :
The shelf life is related to many factors, storage state, buffer ingredients, storage termperature and the stability of the protein itself. Generallly, the shelf life of liquid form is 6 months at -20°C, -80°C. The shelf life of lyophilized form is 12 months at -20°C, -80°C. Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
other info1 :
Calculated MW: 56.1 kDa. Tag Info: N-terminal 6 x His-Tagged. Immunogen Description: Expression Region: 40-520aa Sequene Info: Full length
products description :
Recombinant Protein . 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.
ncbi acc num :
NP_000427.1
ncbi gb acc num :
NM_000436.3
ncbi pathways :
Butanoate Metabolism Pathway (83007); Butanoate Metabolism Pathway (391); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (1270010); Ketone Body Metabolism Pathway (1270026); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Synthesis And Degradation Of Ketone Bodies Pathway (198768); Synthesis And Degradation Of Ketone Bodies Pathway (82936); Synthesis And Degradation Of Ketone Bodies Pathway (297); Utilization Of Ketone Bodies Pathway (1270028)
ncbi summary :
This gene encodes a member of the 3-oxoacid CoA-transferase gene family. The encoded protein is a homodimeric mitochondrial matrix enzyme that plays a central role in extrahepatic ketone body catabolism by catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate. Mutations in this gene are associated with succinyl CoA:3-oxoacid CoA transferase deficiency. [provided by RefSeq, Jul 2008]
uniprot summary :
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. Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; EC 2.8.3.5; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; Transferase. Chromosomal Location of Human Ortholog: 5p13.1. Cellular Component: mitochondrial matrix; mitochondrion; nucleoplasm. Molecular Function: 3-oxoacid CoA-transferase activity. Biological Process: ketone body catabolic process; ketone body metabolic process. Disease: Succinyl-coa:3-oxoacid Coa Transferase Deficiency