Rat BCKDK ELISA Kit | BCKDK elisa kit

Rat BCKDK ELISA Kit

Cat. Num. AAA7606768

• Gene Names: BCKDK; BDK; BCKDKD
• Reactivity: Rat
• Synonyms: BCKDK; Rat BCKDK ELISA Kit; BCKDK elisa kit

• Reactivity: Rat
• Sequence Length: 412

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 78-5000pg/ml
• Sensitivity: 46.9pg/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for BCKDK elisa kit

Principle of the Assay: This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti- [3- methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, Mitochondrial antibody was precoated onto 96-well plates. And the biotin conjugated anti-[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, Mitochondrial antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the [3- methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, Mitochondrial amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, Mitochondrial can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 49457139
• NCBI GeneID: 10295
• UniProt Accession #: O14874; Q6FGL4; Q96G95; Q96IN5; A8MY43
• Molecular Weight: 41,090 Da
• NCBI Official Full Name: BCKDK, partial
• NCBI Official Synonym Full Names: branched chain ketoacid dehydrogenase kinase
• NCBI Official Symbol: BCKDK
• NCBI Official Synonym Symbols: BDK; BCKDKD
• NCBI Protein Information: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; 3-methyl-2-oxobutanoate dehydrogenase [lipoamide] kinase, mitochondrial
• UniProt Protein Name: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
• Protein Family: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase
• UniProt Gene Name: BCKDK
• UniProt Synonym Gene Names: BCKD-kinase; BCKDHKIN

NCBI Description

The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]

Uniprot Description

BCKDK: an atypical protein kinase associated with the mitochondrial matrix. Contains a HATPase_c domain, found in several ATP-binding proteins including protein histidine kinases (PHKs), PHDKs, DNA gyrase B, topoisomerases, heat shock proteins, and DNA mismatch repair proteins. Unlike PHKs, BCK dimerizes through direct interaction of two opposing nucleotide-binding domains. Phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Inactivating mutations have been discovered in BCKDK in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation.

Protein type: ATYPICAL group; EC 2.7.11.4; Kinase, protein; Mitochondrial; PDHK family; Protein kinase, atypical

Chromosomal Location of Human Ortholog: 16p11.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion

Molecular Function: [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; ATP binding; kinase activity; protein binding; protein serine/threonine kinase activity

Biological Process: branched chain family amino acid catabolic process; phosphorylation

Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency