Product Name	DLD
Chinese Name	硫辛酰胺脱氢酶抗体
Alias	DLDH_HUMAN; Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein; GCSL; LAD; PHE3;
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, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	50kDa
Cellular localization	The nucleus cytoplasmic Mitochondrion
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Lipoamide Dehydrogenase: 241-340/509
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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. SWISS: P09622 Gene ID: 1738 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
Product Picture	Paraformaldehyde-fixed, paraffin embedded (rat heart tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (DLDD) Polyclonal Antibody, Unconjugated (SL18295R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.

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