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Home > Product > Antibody > Rabbit Anti-DLDD/Lipoamide Dehydrogenase/FITC Conjugated antibody
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 pyruvat
Cat:
SL18295R-FITC
Species Reactivity：
Rat,(predicted: Human,Mouse,Dog,Cow,Horse,Sheep,Xenopus laevis)
Immunogen：
KLH conjugated synthetic peptide derived from human DLDD/Lipoamide Dehydrogenase
Format：
Lyophilized or Liquid
Storage instructions：
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 ant
Buffer：
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration：
1mg/ml
Clonality：
Polyclonal
Isotype：
IgG
Applications：
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host：
Rabbit
Calculated MW：
50kDa
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Product PDFs
Datasheet:


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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