OXCT; SCOT; Succinyl-CoA:3-Ketoacid-Coenzyme A Transferase 1, Mitochondrial; Somatic-type succinyl-CoA:3-oxoacid CoA-transferase

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against OXCT1. It has been selected for its ability to recognize OXCT1 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Lane1: Human Lung Tissue; Lane2: Rat Brain Tissue; Lane3: Rat Heart Tissue; Lane4: Rat Kidney Tissue.

Western Blot: Sample: Recombinant OXCT1, Human.

DAB staining on IHC-P; Samples: Human Stomach Tissue.

DAB staining on IHC-P; Samples: Human Brain Tissue

DAB staining on IHC-P; Samples: Human Kidney Tissue

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]

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: 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; protein homodimerization activity

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

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

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