HMGCS2 Antibody (PACO56832)
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주문- SKU:
- PACO56832
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | HMGCS2 Antibody (PACO56832) |
Antibody SKU: | PACO56832 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:200-1:500, IF:1:50-1:200 |
Species Reactivity: | Human, Rat |
Immunogen: | Recombinant Human Hydroxymethylglutaryl-CoA synthase, mitochondrial protein (426-508AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western Blot. Positive WB detected in: Rat kidney tissue. All lanes: HMGCS2 antibody at 4.5µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 57, 53, 51 kDa. Observed band size: 57 kDa. |
![]() | IHC image of PACO56832 diluted at 1:400 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
![]() | IHC image of PACO56832 diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
Background: | This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. |
Synonyms: | Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMG-CoA synthase) (EC 2.3.3.10) (3-hydroxy-3-methylglutaryl coenzyme A synthase), HMGCS2 |
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 |
Antibodies |
Anti-HMGCS2 Antibody (CAB14244) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |