protein

DLD, 36-509aa, Human, His tag, E Coli

MBS203646

0.1 mg

285 USD

Recombinant Protein

DLD, 36-509aa, Human, His tag, E Coli

DLD

DLDH; E3; GCSL; LAD; PHE3; Dihydrolipoyl dehydrogenase; mitochondrial Diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase; Dihydrolipoyl dehydrogenase mitochondrial; DLD; E3 component of pyruvate dehydrogenase; E3 component of pyruvate dehydrogenase complex 2 oxo glutarate complex branched chain keto acid dehydrogenase complex; Glycine cleavage system L protein; Glycine cleavage system protein L; Lipoamide reductase; Lipoyl dehydrogenase; PHE 3

dihydrolipoyl dehydrogenase, mitochondrial isoform 1; Dihydrolipoyl dehydrogenase, mitochondrial; dihydrolipoyl dehydrogenase, mitochondrial; E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex; diaphorase; glycine cleavage system L protein; glycine cleavage system protein L; lipoamide dehydrogenase; lipoamide reductase; dihydrolipoamide dehydrogenase; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein

DLD

DLD; DLD; E3; LAD; DLDD; DLDH; GCSL; PHE3; GCSL; LAD; PHE3

DLDH_HUMAN

509

MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSMADQ PIDADVTVIG SGPGGYVAAI KAAQLGFKTV CIEKNETLGG TCLNVGCIPS KALLNNSHYY HMAHGKDFAS RGIEMSEVRL NLDKMMEQKS TAVKALTGGI AHLFKQNKVV HVNGYGKITG KNQVTATKAD GGTQVIDTKN ILIATGSEVT PFPGITIDED TIVSSTGALS LKKVPEKMVV IGAGVIGVEL GSVWQRLGAD VTAVEFLGHV GGVGIDMEIS KNFQRILQKQ GFKFKLNTKV TGATKKSDGK IDVSIEAASG GKAEVITCDV LLVCIGRRPF TKNLGLEELG IELDPRGRIP VNTRFQTKIP NIYAIGDVVA GPMLAHKAED EGIICVEGMA GGAVHIDYNC VPSVIYTHPE VAWVGKSEEQ LKEEGIEYKV GKFPFAANSR AKTNADTDGM VKILGQKSTD RVLGAHILGP GAGEMVNEAA LALEYGASCE DIARVCHAHP TLSEAFREAN LAASFGKSIN F

> 95% by SDS - PAGE

Liquid. In 20 mM Tris-HCl buffer (pH8.0) containing 1mM DTT, 0.1M NaCl, 10% glycerol

1 mg/ml (determined by Bradford assay)

Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.

SDS-PAGE

Antigen Species: Human. Tag: His-tag

Expression System: E Coli

Metabolism

DLD (Dihydrolipoamide dehydrogenase), also known as GCSL (glycine cleavage system L protein), is a component of the glycine cleavage system as well as of the alpha ketoacid dehydrogenase complexes. DLD is a flavin-dependent oxidoreductase and functions as a component of the alpha-keto acid dehydrogenase, the pyruvate dehydrogenase, the alpha-ketoglutarate dehydrogenase, the branched-chain alpha-keto acid dehydrogenase and as the L protein in the mitochondrial glycine cleavage system. Mutations in DLD protein can result in MSuD (maple syrup urine disease) and congenital infantile lactic acidosis. Recombinant human DLD protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Giannopoulou E., et al. (2009) Anticancer Res. 29(12):5077-82. Daee DL., et al. (2009) Proc Natl Acad Sci u S A. 107(1):157-62.

91199540

NP_000099

NM_000108.4

54.4 kDa (511aa)

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); Branched-chain Amino Acid Catabolism Pathway (106179); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Citrate Cycle (TCA Cycle) Pathway (82927)

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]

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: EC 1.8.1.4; Mitochondrial; Oxidoreductase; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Amino Acid Metabolism - glycine, serine and threonine; Carbohydrate Metabolism - citrate (TCA) cycle; Amino Acid Metabolism - valine, leucine and isoleucine degradation. 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; gastrulation; regulation of acetyl-CoA biosynthetic process from pyruvate; branched chain family amino acid catabolic process; proteolysis; pyruvate metabolic process; sperm capacitation. Disease: Dihydrolipoamide Dehydrogenase Deficiency

0.1 mg

285 USD

0.5 mg

640