ELISA/assay

Human Dihydrolipoyl dehydrogenase, mitochondrial ELISA Kit

MBS2885103

48-Strip-Wells

400 USD

ELISA Kit

Human Dihydrolipoyl dehydrogenase, mitochondrial ELISA Kit

[Dihydrolipoyl dehydrogenase, mitochondrial]

[Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein; DLD; GCSL; LAD; PHE3; 1.8.1.4]

[dihydrolipoyl dehydrogenase, mitochondrial isoform 1; Dihydrolipoyl dehydrogenase, mitochondrial; dihydrolipoyl dehydrogenase, mitochondrial; dihydrolipoamide dehydrogenase; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein]

[DLD]

[DLD; DLD; E3; LAD; DLDD; DLDH; GCSL; PHE3; GCSL; LAD; PHE3]

DLDH_HUMAN

Human

509

For long term storage, please store the entire kit at -20 degree C.

Typical Testing Data/Standard Curve (for reference only)

Assay Type: Competitive. Detection Range: 0.78-50 ng/mL. Sensitivity: 0.45 ng/mL

Intra-Assay CV: <=8.4%. Inter-Assay CV: <=11.1%. Recovery: 91%

Intended Uses: This immunoassay kit allows for the in vitro quantitative determination of target antigen concentrations in serum, plasma, tissue homogenates, cell culture supernates or other biological fluids. Principle of the Assay: The ELISA is based on the competitive binding enzyme immunoassay technique. The microtiter plate provided in this kit has been pre-coated with an antibody specific to target antigen, During the reaction, target antigen in the sample or standard competes with a fixed amount of biotin-labeled target antigen for sites on a pre-coated antibody specific to target antigen. Excess conjugate and unbound sample or standard are washed from the plate. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then a TMB substrate solution is added to each well. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The concentration of target antigen in the samples is then determined by comparing the O.D. of the samples to the standard curve.

91199540

NP_000099.2

NM_000108.4

P09622

49,283 Da

2-oxobutanoate Degradation Pathway (142413); 2-oxobutanoate Degradation I Pathway (139464); 2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway (142336); 2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway (139119); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (142335); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (139118); Branched-chain Amino Acid Catabolism Pathway (1270161); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Citrate Cycle (TCA Cycle) Pathway (82927)

This gene encodes a member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]

DLD: a multi-functional mitochondrial enzyme. An enzymatic component of the mitochondrial glycine cleavage system, the pyruvate dehydrogenase complex (PDHC), the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. Is the E3 component of the PDHC that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. The E3 component has dihydrolipoamide dehydrogenase activity. The PDHC contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. Defects in DLD are a cause of maple syrup urine disease (MSUD), characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation. Differentially expressed in the Wernicke's Area from patients with schizophrenia. Inhibited by 5-methoxyindole-2-carboxylic acid (MICA). Protein type: Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - pyruvate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Amino Acid Metabolism - glycine, serine and threonine; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.8.1.4. Chromosomal Location of Human Ortholog: 7q31-q32. Cellular Component: acrosomal matrix; cilium; mitochondrial matrix; mitochondrion; myelin sheath; nucleoplasm; oxoglutarate dehydrogenase complex; pyruvate dehydrogenase complex. Molecular Function: dihydrolipoyl dehydrogenase activity; FAD binding; lipoamide binding; NAD binding. Biological Process: 2-oxoglutarate metabolic process; aging; branched chain family amino acid catabolic process; cell redox homeostasis; cellular metabolic process; dihydrolipoamide metabolic process; gastrulation; glyoxylate metabolic process; lipoate metabolic process; lysine catabolic process; mitochondrial electron transport, NADH to ubiquinone; proteolysis; pyruvate metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; regulation of membrane potential; sperm capacitation; tricarboxylic acid cycle. Disease: Dihydrolipoamide Dehydrogenase Deficiency

48-Strip-Wells

400 USD

96-Strip-Wells

530

5x96-Strip-Wells

2305

10x96-Strip-Wells

4250