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Dihydrolipoyl Dehydrogenase (DLD)

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LAD; GCSL; Dihydrolipoamide Dehydrogenase; E3 Component Of Pyruvate Dehydrogenase Complex; 2-Oxo-Glutarate Complex; Branched Chain Keto Acid Dehydrogenase Complex

Dihydrolipoyl Dehydrogenase (DLD)
CCC A919
HGNC 2898
MGI 107450
PubMed 17110466
RGD 735073
UniProt P09622
Wiki DLD

The dihydrolipoate, still bound to a lysine residue of the complex, then migrates to the dihydrolipoyl dehydrogenase (E3) active site where it undergoes a flavin-mediated oxidation, identical in chemistry to disulfide isomerase. First, FAD oxidizes dihydrolipoate back to its lipoate resting state, producing FADH2.

Dihydrolipoamide dehydrogenase is the L protein of the mitochondrial glycine cleavage system. The L protein, also named dihydrolipoamide dehydrogenase, is also a component of the pyruvate dehydrogenase complex, the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA919Hu01 Recombinant Dihydrolipoyl Dehydrogenase (DLD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA919Hu01 Polyclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) WB; IHC; ICC/IF
MAA919Hu22 Monoclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Dihydrolipoyl Dehydrogenase (DLD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoyl Dehydrogenase (DLD) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA919Mu01 Recombinant Dihydrolipoyl Dehydrogenase (DLD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA919Mu01 Polyclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Dihydrolipoyl Dehydrogenase (DLD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoyl Dehydrogenase (DLD) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA919Ra01 Recombinant Dihydrolipoyl Dehydrogenase (DLD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA919Ra01 Polyclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Dihydrolipoyl Dehydrogenase (DLD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoyl Dehydrogenase (DLD) ELISA Kit Customized Service Offer

Organism species: Canis familiaris; Canine (Dog)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dihydrolipoyl Dehydrogenase (DLD) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dihydrolipoyl Dehydrogenase (DLD) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dihydrolipoyl Dehydrogenase (DLD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoyl Dehydrogenase (DLD) ELISA Kit Customized Service Offer
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  18. "Molecular basis of lipoamide dehydrogenase deficiency in Ashkenazi Jews."Am. J. Med. Genet. 82:177-182(1999) [PubMed] [Europe PMC] [Abstract]