Anti-OXCT1/SCOT Antibody Picoband® Fluoro488 Conjugated

3-oxoacid CoA-transferase 1 (OXCT1) is an enzyme that in humans is encoded by the OXCT1 gene. It is also known as succinyl-CoA-3-oxaloacid CoA transferase (SCOT) . 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.

DNA- (apurinic or apyrimidinic site) lyase 2;3.1.-.-;4.2.99.18; AP endonuclease XTH2; APEX nuclease 2; APEX nuclease-like 2; Apurinic-apyrimidinic endonuclease 2; AP endonuclease 2; APEX2; APE2, APEXL2, XTH2

Function as a weak apurinic/apyrimidinic (AP) endodeoxyribonuclease in the DNA base excision repair (BER) pathway of DNA lesions induced by oxidative and alkylating agents. Initiates repair of AP sites in DNA by catalyzing hydrolytic incision of the phosphodiester backbone immediately adjacent to the damage, generating a single-strand break with 5'-deoxyribose phosphate and 3'-hydroxyl ends. Displays also double-stranded DNA 3'-5' exonuclease, 3'-phosphodiesterase activities. Shows robust 3'-5' exonuclease activity on 3'-recessed heteroduplex DNA and is able to remove mismatched nucleotides preferentially. Shows fairly strong 3'-phosphodiesterase activity involved in the removal of 3'-damaged termini formed in DNA by oxidative agents. In the nucleus functions in the PCNA-dependent BER pathway. Required for somatic hypermutation (SHM) and DNA cleavage step of class switch recombination (CSR) of immunoglobulin genes. Required for proper cell cycle progression during proliferation of peripheral lymphocytes. .

1. Fukao, T., Mitchell, G. A., Song, X.-Q., Nakamura, H., Kassovska-Bratinova, S., Orii, K. E., Wraith, J. E., Besley, G., Wanders, R. J. A., Niezen-Koning, K. E., Berry, G. T., Palmieri, M., Kondo, N. Succinyl-CoA:3-ketoacid CoA transferase (SCOT) : cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations. Genomics 68: 144-151, 2000. 2. Hori, T., Fukao, T., Murase, K., Sakaguchi, N., Harding, C. O., Kondo, N. Molecular basis of two-exon skipping (exons 12 and 13) by c.1248+5g-a in OXCT1 gene: study on intermediates of OXCT1 transcripts in fibroblasts. Hum. Mutat. 34: 473-480, 2013. 3. Kassovska-Bratinova, S., Fukao, T., Song, X.-Q., Duncan, A. M. V., Chen, H. S., Robert, M.-F., Perez-Cerda, C., Ugarte, M., Chartrand, C., Vobecky, S., Kondo, N., Mitchell, G. A. Succinyl CoA:3-oxoacid CoA transferase (SCOT) : human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient. Am. J. Hum. Genet. 59: 519-528, 1996.