Cell Biology Antibodies 17

Anti-DLDH Antibody (CAB5220)

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SKU:
CAB5220
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-DLDH Antibody
Antibody SKU:CAB5220
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human DLDH
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:SKOV3, U-251MG, Mouse liver, Mouse brain, Rat brain
Immunogen:A synthesized peptide derived from human DLDH
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:1738
Uniprot:P09622
Cellular Location:
Calculated MW:56kDa
Observed MW:60KDa
Synonyms:DLDD, DLDH, E3, GCSL, LAD, PHE3
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]
UniProt Protein Function: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).
UniProt Protein Details:

Protein type:Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Mitochondrial; EC 1.8.1.4; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - citrate (TCA) cycle

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: cellular metabolic process; regulation of membrane potential; mitochondrial electron transport, NADH to ubiquinone; cell redox homeostasis; tricarboxylic acid cycle; detoxification of mercury ion; lysine catabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; gastrulation; branched chain family amino acid catabolic process; proteolysis; pyruvate metabolic process; sperm capacitation

Disease: Dihydrolipoamide Dehydrogenase Deficiency

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 Code:P09622
NCBI GenInfo Identifier:269849557
NCBI Gene ID:1738
NCBI Accession:P09622.2
UniProt Secondary Accession:P09622,Q14131, Q14167, Q59EV8, Q8WTS4, B2R5X0, B4DHG0 B4DT69,
UniProt Related Accession:P09622
Molecular Weight:509
NCBI Full Name:Dihydrolipoyl dehydrogenase, mitochondrial
NCBI Synonym Full Names:dihydrolipoamide dehydrogenase
NCBI Official Symbol:DLD  
NCBI Official Synonym Symbols:E3; LAD; DLDD; DLDH; GCSL; PHE3  
NCBI Protein Information:dihydrolipoyl dehydrogenase, mitochondrial; diaphorase; lipoamide reductase; lipoamide dehydrogenase; glycine cleavage system L protein; glycine cleavage system protein L; E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex
UniProt Protein Name:Dihydrolipoyl dehydrogenase, mitochondrial
UniProt Synonym Protein Names:Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein
Protein Family:Delta-like protein
UniProt Gene Name:DLD  
UniProt Entry Name:DLDH_HUMAN
Anti-DLDH Antibody (CAB5220)Western blot - DLDH Rabbit mAb (CAB5220)
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