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Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2)

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3-hydroxy-3-methylglutaryl coenzyme A synthase

Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2)
CCC D687
HGNC 5008
MGI 101939
PubMed 16940161
RGD 2804
UniProt P54868
Wiki HMGCS2
HMGCS2 belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase (HMGCS2) mediates the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for brain, heart, kidney, and other organs during times of carbohydrate deprivation such as fasting .

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Polyclonal Antibody Customized Service Offer
Assay Kits SED687Hu ELISA Kit for Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD687Mu01 Recombinant Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAD687Mu01 Polyclonal Antibody to Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) CLIA Kit Customized Service Offer
n/a ELISA Kit for Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Polyclonal Antibody Customized Service Offer
Assay Kits SED687Ra ELISA Kit for Hydroxymethylglutaryl Coenzyme A Synthase 2, Mitochondrial (HMGCS2) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Molecular cloning and tissue expression of human mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase."Arch. Biochem. Biophys. 317:385-390(1995) [PubMed] [Europe PMC] [Abstract]
  2. "Cloning and characterization of the human mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase gene."Gene 195:121-126(1997) [PubMed] [Europe PMC] [Abstract]
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  4. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
  5. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  6. "Human mitochondrial HMG CoA synthase: liver cDNA and partial genomic cloning, chromosome mapping to 1p12-p13, and possible role in vertebrate evolution."Genomics 23:552-559(1994) [PubMed] [Europe PMC] [Abstract]
  7. "Characterization of splice variants of the genes encoding human mitochondrial HMG-CoA lyase and HMG-CoA synthase, the main enzymes of the ketogenesis pathway."Mol. Biol. Rep. 39:4777-4785(2012) [PubMed] [Europe PMC] [Abstract]
  8. "Ketogenic HMGCS2 Is a c-Myc target gene expressed in differentiated cells of human colonic epithelium and down-regulated in colon cancer."Mol. Cancer Res. 4:645-653(2006) [PubMed] [Europe PMC] [Abstract]
  9. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  10. "Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design."J. Mol. Biol. 398:497-506(2010) [PubMed] [Europe PMC] [Abstract]
  11. "Genetic basis of mitochondrial HMG-CoA synthase deficiency."Hum. Genet. 109:19-23(2001) [PubMed] [Europe PMC] [Abstract]
  12. "Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency: clinical course and description of causal mutations in two patients."Pediatr. Res. 49:326-331(2001) [PubMed] [Europe PMC] [Abstract]
  13. "Mitochondrial HMG-CoA synthase deficiency: identification of two further patients carrying two novel mutations."Eur. J. Pediatr. 162:279-280(2003) [PubMed] [Europe PMC] [Abstract]