Product Name	Anti-DLDD/Lipoamide Dehydrogenase/AF594
Chinese Name	AF594标记的硫辛酰胺脱氢酶抗体
Alias	Dehydrogenase complex, E3 component; Diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase; Dihydrolipoyl dehydrogenase mitochondrial; dld; DLDD; DLDH; DLDH_HUMAN; E3; E3 branched chain aplha-keto acid; E3 component of pyruvate dehydrogenase; E3 component of pyruvate dehydrogenase complex 2 oxo glutarate complex branched chain keto acid dehydrogenase complex; GCSL; Glycine cleavage system L protein; Glycine cleavage system protein L; LAD; lipoamide dehydrogenase; Lipoamide reductase; Lipoyl dehydrogenase; mitochondrial; OTTHUMP00000206744; OTTHUMP00000206746; OTTHUMP00000206748; OTTHUMP00000206749; PHE 3; PHE3; Pyruvate dehydrogenase component E3.
Research Area	Tumour  Cell biology  Signal transduction  The new supersedes the old  Mitochondrion
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Rat,  (predicted: Human, Mouse, Dog, Cow, Horse, Sheep, Xenopus laevis)
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	50kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human DLDD/Lipoamide Dehydrogenase
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: 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] Function: Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction. Subcellular Location: Mitochondrion matrix. Post-translational modifications: Tyrosine phosphorylated. DISEASE: Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis. Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600]. MSUD is 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. Similarity: Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. Database links: Entrez Gene: 1738 Human Entrez Gene: 403978 Dog Entrez Gene: 13382 Mouse Entrez Gene: 397129 Pig Entrez Gene: 298942 Rat Omim: 238331 Human SwissProt: P49819 Dog SwissProt: P09622 Human SwissProt: O08749 Mouse SwissProt: P09623 Pig SwissProt: Q6P6R2 Rat Unigene: 131711 Human Unigene: 3131 Mouse Unigene: 86962 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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