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Rabbit Anti-DLDD/Lipoamide Dehydrogenase/BF350 Conjugated antibody (bs-18295R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-18295R-BF350
英文名稱 Rabbit Anti-DLDD/Lipoamide Dehydrogenase/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的硫辛酰胺脫氫酶抗體
別    名 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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat,  (predicted: Human, Mouse, Dog, Cow, Horse, Sheep, Xenopus laevis)
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 50kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DLDD/Lipoamide Dehydrogenase
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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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