HMGCS2 encodes 3-hydroxy-3-methylglutaryl-CoA synthase 2, a mitochondrial enzyme also known as mitochondrial HMG-CoA synthase (mHS). It catalyzes the rate-limiting step in ketogenesis: condensing acetyl-CoA and acetoacetyl-CoA to form HMG-CoA, which is critical for producing ketone bodies as an alternative energy source during fasting, prolonged exercise, or low-carbohydrate intake.
Dysregulation of HMGCS2 links to fatty acid oxidation and ketone metabolism pathways. Mutations cause HMGCS2 deficiency, a rare autosomal recessive disorder with hypoketotic hypoglycemia and metabolic acidosis. Therapeutic research targets HMGCS2 to enhance ketogenesis for epilepsy (aligning with ketogenic diet effects) and neurodegenerative diseases; preclinical agonists show neuroprotective potential, though no FDA-approved drugs exist yet.
Recombinant Human Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) (CSB-EP010567HU)
Validated Data
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
HMGCS2 Recombinant Monoclonal Antibody (CSB-RA693900A0HU)
Validated Data
Western Blot
Positive WB detected in: A549 whole cell lysate, Ntera-2 whole cell lysate
All lanes: HMGCS2 antibody at 1:500
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 57, 53, 51 kDa
Observed band size: 55 kDa
IHC image of CSB-RA693900A0HU diluted at 1:100 and staining in paraffin-embedded human small intestine 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
IHC image of CSB-RA693900A0HU diluted at 1:100 and staining in paraffin-embedded human appendix 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
HMGCS2 Antibody (CSB-PA010567LA01HU)
Validated Data
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 CSB-PA010567LA01HU 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.
IHC image of CSB-PA010567LA01HU 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.
Immunofluorescence staining of HepG2 cells with CSB-PA010567LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
The following HMGCS2 reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
HMGCS2 Antibodies for Homo sapiens (Human)
| Code | Product Name | Species Reactivity | Application |
|---|---|---|---|
| CSB-PA187322 | HMGCS2 Antibody | Human | ELISA,WB,IHC |
| CSB-PA045049 | HMGCS2 Antibody | Human,Mouse | ELISA,WB,IHC |
| CSB-PA010567LA01HU | HMGCS2 Antibody | Human, Rat | ELISA, WB, IHC, IF |
| CSB-PA010567LB01HU | HMGCS2 Antibody, HRP conjugated | Human | ELISA |
| CSB-PA010567LC01HU | HMGCS2 Antibody, FITC conjugated | Human | |
| CSB-PA010567LD01HU | HMGCS2 Antibody, Biotin conjugated | Human | ELISA |
| CSB-RA693900A0HU | HMGCS2 Recombinant Monoclonal Antibody | Human | ELISA, WB, IHC |
HMGCS2 Proteins for Rattus norvegicus (Rat)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP010567RA CSB-EP010567RA CSB-BP010567RA CSB-MP010567RA CSB-EP010567RA-B |
Recombinant Rat Hydroxymethylglutaryl-CoA synthase, mitochondrial (Hmgcs2) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
HMGCS2 Proteins for Homo sapiens (Human)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP010567HU CSB-BP010567HU CSB-MP010567HU CSB-EP010567HU-B |
Recombinant Human Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP010567HU | Recombinant Human Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) | E.coli |
HMGCS2 Proteins for Mus musculus (Mouse)
| Code | Product Name | Source |
|---|---|---|
| CSB-BP010567MO CSB-MP010567MO CSB-EP010567MO-B |
Recombinant Mouse Hydroxymethylglutaryl-CoA synthase, mitochondrial (Hmgcs2) | Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
HMGCS2 Proteins for Sus scrofa (Pig)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP010567PI CSB-EP010567PI CSB-BP010567PI CSB-MP010567PI CSB-EP010567PI-B |
Recombinant Pig Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
HMGCS2 Proteins for Bos taurus (Bovine)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP643446BO CSB-EP643446BO CSB-BP643446BO CSB-MP643446BO CSB-EP643446BO-B |
Recombinant Bovine Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |