catalog number :
MBS9403435
products full name :
DLD Antibody
products short name :
[DLD]
products name syn :
[E3; LAD; DLDD; DLDH; GCSL]
other names :
[dihydrolipoyl dehydrogenase, mitochondrial isoform 1; Dihydrolipoyl dehydrogenase, mitochondrial; dihydrolipoyl dehydrogenase, mitochondrial; dihydrolipoamide dehydrogenase; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein]
products gene name :
[DLD]
other gene names :
[DLD; DLD; E3; LAD; DLDD; DLDH; GCSL; PHE3; GCSL; LAD; PHE3]
uniprot entry name :
DLDH_HUMAN
reactivity :
Human, Mouse, Rat
specificity :
The antibody detects endogenous level of total DLD protein.
purity :
Antibodies were purified by affinity purification using immunogen.
form :
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
concentration :
1.0 mg/ml
storage stability :
Store at -20°C
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
app notes :
Western blotting: 1:500 - 1:2000. Immunohistochemistry: 1:50 - 1:200. Immunofluorescence: 1:50 - 1:200
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunohistochemistry (IHC)
image4 heading :
Immunohistochemistry (IHC)
image4 description :
Immunohistochemical analysis of paraffin-embedded human stomach using DLD antibody at dilution of 1:200 (200x lens).
image5 heading :
Immunohistochemistry (IHC)
image5 description :
Immunohistochemical analysis of paraffin-embedded human stomach using DLD antibody at dilution of 1:200 (400x lens).
other info1 :
Immunogen Type: Recombinant Protein. Immunogen Description: Recombinant protein of human DLD. SDS-PAGE MW: 54kD
other info2 :
Target Name: DLD
products categories :
Total protein Ab
products description :
This gene encodes 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.
ncbi acc num :
NP_000099.2
ncbi gb acc num :
NM_000108.4
ncbi pathways :
2-oxobutanoate Degradation Pathway (142413); 2-oxobutanoate Degradation I Pathway (139464); 2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway (142336); 2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway (139119); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (142335); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (139118); Biosynthesis Of Antibiotics Pathway (1144997); Branched-chain Amino Acid Catabolism Pathway (1270161); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567)
ncbi summary :
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]
uniprot summary :
DLD: a multi-functional mitochondrial enzyme. An enzymatic component of the mitochondrial glycine cleavage system, the pyruvate dehydrogenase complex (PDHC), the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. Is the E3 component of the PDHC that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. The E3 component has dihydrolipoamide dehydrogenase activity. The PDHC contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. Defects in DLD are a cause of maple syrup urine disease (MSUD), 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. Differentially expressed in the Wernicke s Area from patients with schizophrenia. Inhibited by 5-methoxyindole-2-carboxylic acid (MICA). Protein type: Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - citrate (TCA) cycle; Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Mitochondrial; EC 1.8.1.4; Carbohydrate Metabolism - pyruvate. Chromosomal Location of Human Ortholog: 7q31-q32. Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; acrosomal matrix; cilium. Molecular Function: mercury (II) reductase activity; FAD binding; mercury ion binding; dihydrolipoyl dehydrogenase activity; NADP binding. Biological Process: mitochondrial electron transport, NADH to ubiquinone; tricarboxylic acid cycle; gastrulation; regulation of acetyl-CoA biosynthetic process from pyruvate; branched chain family amino acid catabolic process; proteolysis; cellular metabolic process; regulation of membrane potential; cell redox homeostasis; detoxification of mercury ion; lysine catabolic process; pyruvate metabolic process; sperm capacitation. Disease: Dihydrolipoamide Dehydrogenase Deficiency