3-Oxoacid CoA transferase 1 (OCT1) is a mitochondrial enzyme that catalyzes the transfer of coenzyme A from succinyl-CoA to acetoacetate during ketone body catabolism. Mutations in OXCT1 have been linked to Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency. Rabbit Anti-OXCT1 antibody recognizes pig, bovine, chicken, human, mouse, and rat,OXCT1.
Immunogen
Synthetic peptide directed towards the middle region of human OXCT1
Application
Rabbit Anti-OXCT1 (AB2) antibody is suitable for western blot applications at a concentration of 1 μg/ml.
Biochem/physiol Actions
CT1 is a member of the 3-oxoacid CoA-transferase gene family. It 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.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.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.
Sequence
Synthetic peptide located within the following region: GMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLIN
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Journal of inherited metabolic disease, 33 Suppl 3, S307-S313 (2010-07-24)
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency causes episodic ketoacidotic crises and no apparent symptoms between them. Here, we report a Japanese case of neonatal-onset SCOT deficiency. The male patient presented a severe ketoacidotic crisis, with blood pH of 7.072 and bicarbonate
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