Mouse Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) ELISA Kit | HMGCS2 elisa kit

Mouse Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2) ELISA Kit

Cat. Num. AAA7204372

• Reactivity: Mouse
• Synonyms: Hydroxymethylglutaryl-CoA synthase; mitochondrial (HMGCS2); Mouse Hydroxymethylglutaryl-CoA synthase; mitochondrial (HMGCS2) ELISA Kit; HMGCS2 elisa kit

• Reactivity: Mouse
• Specificity: This assay has high sensitivity and excellent specificity for detection of HMGCS2. No significant cross-reactivity or interference between HMGCS2 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between HMGCS2 and all the analogues, therefore, cross reaction may still exist in some cases.

• Samples: Serum, plasma, cell culture supernatants, body fluid and tissue homogenate
• Assay Type: Quantitative Competitive
• Sensitivity: 1.0 pg/mL
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for HMGCS2 elisa kit

Intended Uses: This HMGCS2 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Mouse HMGCS2. This ELISA kit for research use only, not for therapeutic or test applications!

Principle of the Assay: HMGCS2 ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-HMGCS2 antibody and an HMGCS2-HRP conjugate. The assay sample and buffer are incubated together with HMGCS2-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the HMGCS2 concentration since HMGCS2 from samples and HMGCS2-HRP conjugate compete for the anti-HMGCS2 antibody binding site. Since the number of sites is limited, as more sites are occupied by HMGCS2 from the sample, fewer sites are left to bind HMGCS2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The HMGCS2 concentration in each sample is interpolated from this standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 260656028
• NCBI GeneID: 3158
• NCBI Accession #: NP_001159579.1
• NCBI GenBank Nucleotide #: NM_001166107.1
• Molecular Weight: 50,050 Da
• NCBI Official Full Name: hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 2
• NCBI Official Synonym Full Names: 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)
• NCBI Official Symbol: HMGCS2
• NCBI Protein Information: hydroxymethylglutaryl-CoA synthase, mitochondrial; HMG-CoA synthase; 3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
• UniProt Protein Name: Hydroxymethylglutaryl-CoA synthase, mitochondrial
• UniProt Gene Name: HMGCS2
• UniProt Synonym Gene Names: HMG-CoA synthase
• UniProt Entry Name: HMCS2_HUMAN

NCBI Description

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 Description

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.

Protein type: Lipid Metabolism - synthesis and degradation of ketone bodies; Carbohydrate Metabolism - butanoate; Transferase; Mitochondrial; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; EC 2.3.3.10; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 1p13-p12

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane

Molecular Function: hydroxymethylglutaryl-CoA synthase activity

Biological Process: isoprenoid biosynthetic process; ketone body biosynthetic process; ketone body metabolic process; cellular lipid metabolic process; cholesterol biosynthetic process

Disease: 3-hydroxy-3-methylglutaryl-coa Synthase 2 Deficiency