Human Pyruvate dehydrogenase E1 component subunit beta, mitochondrial (PDHB) ELISA Kit

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AE28196HU
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Product Name Human Pyruvate dehydrogenase E1 component subunit beta, mitochondrial (PDHB) ELISA Kit
Description Koike et al. (1988) cloned and sequenced cDNAs encoding the alpha and the beta subunits. Theoretically, there may be 2 forms of pyruvate dehydrogenase deficiency, one with mutation in the PDHA gene and one with mutation in the PDHB gene. Ho et al. (1988) isolated a 1.5-kb cDNA clone for the beta subunit of E1 from a human liver gamma-gt11 cDNA library using anti-E1 serum. Using a cDNA probe, Olson et al. (1990) demonstrated that the PHE1B gene is located on 3p13-q23. Koike et al. (1990) described the molecular cloning of the entire human PHE1B gene, its characterization by restriction enzyme analysis, and its complete nucleotide sequence. The gene is composed of 10 exons and 9 introns. All intron-exon splice junctions follow the GT/AG rule. The Alu family was found in introns 2 and 8. This assay has high sensitivity and excellent specificity for detection of Human PDHB. No significant cross-reactivity or interference between Human PDHB and analogues was observed.
Synonyms DKFZp564K0164, PHE1B, Pyruvate dehydrogenase, E1 beta polypeptide
Method Sandwich ELISA
Detection Range 1.56-100 ng/mL
Sensitivity æ0.65 ng/mL
Reactivity Human
Sample Types Serum, Plasma, Other biological fluids.
Background Koike et al. (1988) cloned and sequenced cDNAs encoding the alpha and the beta subunits. Theoretically, there may be 2 forms of pyruvate dehydrogenase deficiency, one with mutation in the PDHA gene and one with mutation in the PDHB gene. Ho et al. (1988) isolated a 1.5-kb cDNA clone for the beta subunit of E1 from a human liver gamma-gt11 cDNA library using anti-E1 serum. Using a cDNA probe, Olson et al. (1990) demonstrated that the PHE1B gene is located on 3p13-q23. Koike et al. (1990) described the molecular cloning of the entire human PHE1B gene, its characterization by restriction enzyme analysis, and its complete nucleotide sequence. The gene is composed of 10 exons and 9 introns. All intron-exon splice junctions follow the GT/AG rule. The Alu family was found in introns 2 and 8.
Supplier Abebio

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