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

Polyclonal

IgG

Rabbit

>=95% as determined by SDS-PAGE
Immunogen Affinity Purified

Liquid

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-OXCT1 antibody 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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), Immunoprecipitation (IP)

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

Chromosomal Location of Human Ortholog: 5p13.1

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: 3-oxoacid CoA-transferase activity; protein homodimerization activity

Biological Process: brain development; heart development; ketone body catabolic process; ketone body metabolic process; ketone catabolic process; response to activity; response to drug; response to ethanol; response to hormone stimulus; response to nutrient; response to starvation

Disease: Succinyl-coa:3-oxoacid Coa Transferase Deficiency

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