Anti-HMGCS2 Antibody (CAB19232)
- SKU:
- CAB19232
- Product type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- HMGCS2
- Hydroxymethylglutaryl-CoA synthase
- mitochondrial
- 3-hydroxy-3-methylglutaryl coenzyme A synthase
- 3-hydroxy-3-methylglutaryl-CoA synthase 2
Frequently bought together:
Description
| Product Name: | HMGCS2 Rabbit mAb |
| Product Code: | CAB19232 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | HMGCS2, Hydroxymethylglutaryl-CoA synthase, mitochondrial, 3-hydroxy-3-methylglutaryl coenzyme A synthase, 3-hydroxy-3-methylglutaryl-CoA synthase 2 |
| Applications: | WB, IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant protein of human HMGCS2. |
| Applications: | WB, IHC |
| Recommended Dilutions: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HepG2, Mouse liver, Rat liver |
| Immunogen: | Recombinant protein of human HMGCS2. |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 3158 |
| Uniprot: | P54868 |
| Cellular Location: | Mitochondrion |
| Calculated MW: | 50kDa |
| Observed MW: | 50KDa |
| UniProt Protein Function: | HMGCS2: This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency); also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids. Belongs to the HMG-CoA synthase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Transferase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.3.10; Carbohydrate Metabolism - butanoate; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Lipid Metabolism - synthesis and degradation of ketone bodies Chromosomal Location of Human Ortholog: 1p13-p12 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:hydroxymethylglutaryl-CoA synthase activity Biological Process: ketone body biosynthetic process Disease: 3-hydroxy-3-methylglutaryl-coa Synthase 2 Deficiency |
| NCBI Summary: | The protein encoded by this gene 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.[provided by RefSeq, Oct 2009] |
| UniProt Code: | P54868 |
| NCBI GenInfo Identifier: | 1708234 |
| NCBI Gene ID: | 3158 |
| NCBI Accession: | P54868.1 |
| UniProt Secondary Accession: | P54868,Q5SZU2, Q6IBF4, B7Z8R3, D3Y5K6, |
| UniProt Related Accession: | P54868 |
| Molecular Weight: | 50,050 Da |
| NCBI Full Name: | Hydroxymethylglutaryl-CoA synthase, mitochondrial |
| NCBI Synonym Full Names: | 3-hydroxy-3-methylglutaryl-CoA synthase 2 |
| NCBI Official Symbol: | HMGCS2 |
| NCBI Protein Information: | hydroxymethylglutaryl-CoA synthase, mitochondrial |
| UniProt Protein Name: | Hydroxymethylglutaryl-CoA synthase, mitochondrial |
| UniProt Synonym Protein Names: | 3-hydroxy-3-methylglutaryl coenzyme A synthase |
| UniProt Gene Name: | HMGCS2 |
| UniProt Entry Name: | HMCS2_HUMAN |
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