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    DLD Rabbit pAb (bs-18295R)  
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    產(chǎn)品編號 bs-18295R
    英文名稱(chēng) DLD Rabbit pAb
    中文名稱(chēng) 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(lǐng)域 腫瘤  細胞生物  信號轉導  新陳代謝  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    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(xiàn)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 30min; Antibody incubation with (DLDD) Polyclonal Antibody, Unconjugated (bs-18295R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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