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DLD Rabbit pAb (bs-18295R)  
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產(chǎn)品編號 bs-18295R
英文名稱 DLD Rabbit pAb
中文名稱 硫辛酰胺脫氫酶抗體
別    名 DLDH_HUMAN; Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein; GCSL; LAD; PHE3;  
研究領域 腫瘤  細胞生物  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,Rat (predicted: Human,Sheep,Cow,Dog,Horse,Xenopus laevis)
產(chǎn)品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 50 kDa
檢測分子量
細胞定位 細胞核 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Lipoamide Dehydrogenase: 241-340/509 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



產(chǎn)品圖片
Sample: Lane 1: Mouse Heart tissue lysates Lane 2: Mouse Testis tissue lysates Primary: Anti-DLD (bs-18295R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 50 kDa Observed band size: 52 kDa
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 30mi
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