Succinyl-CoA:3-ketoacid coenzyme A transferase 1; mitochondrial; 3-oxoacid CoA-transferase 1; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase; SCOT-s; OXCT1; OXCT; SCOT

For Research Use Only. Not for use in diagnostic procedures.

The synthetic peptide sequence is selected from aa 272-286 of HUMAN OXCT1

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of OXCT1 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

13,358 Da

3-oxoacid CoA-transferase 1

succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial

Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial

OXCT; SCOT; SCOT-s??[Similar Products]

SCOT1_HUMAN

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]

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: Mitochondrial; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Transferase; Lipid Metabolism - synthesis and degradation of ketone bodies; EC 2.8.3.5

Chromosomal Location of Human Ortholog: 5p13.1

Cellular Component: mitochondrial matrix; mitochondrion

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

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