Rabbit Anti-HMGCS2 antibody

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3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial); 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2; 3 hydroxy 3 methylglutaryl coenzyme A synthase; 3-hydroxy-3-methylglutaryl coenzyme A synthase; HMCS2_HUMAN; HMG CoA synthase; HMG-CoA synt

Cat:

SL5070R

Species Reactivity：

Mouse,Rat,(predicted: Human,Dog,Pig,Cow,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human HMGCS2:101-200/508

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Tissue/cell: rat colitis tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min; Incubation: Anti-HMGCS2 Polyclonal Antibody, Unconjugated(SL5070R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(SLC0010) staining

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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HMGCS2 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.

Function:
This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.

DISEASE:
Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency) [MIM:605911]; 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.

Similarity:
Belongs to the HMG-CoA synthase family.

SWISS:
P54868

Gene ID:
3158

Database links:
UniProtKB/Swiss-Prot: P54868.1

Picture

Tissue/cell: rat colitis tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min;
Incubation: Anti-HMGCS2 Polyclonal Antibody, Unconjugated(SL5070R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(SLC0010) staining

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