Anti-DLD Antibody (CAB5403)
- SKU:
- CAB5403
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
抗体名: | Anti-DLD Antibody |
抗体コード: | CAB5403 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 36-300 of human DLD (NP_000099.2). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | MCF7, BT-474, K-562, 293T, Mouse kidney, Mouse skeletal muscle, Mouse liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 36-300 of human DLD (NP_000099.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | ADQP IDAD VTVI GSGP GGYV AAIK AAQL GFKT VCIE KNET LGGT CLNV GCIP SKAL LNNS HYYH MAHG KDFA SRGI EMSE VRLN LDKM MEQK STAV KALT GGIA HLFK QNKV VHVN GYGK ITGK NQVT ATKA DGGT QVID TKNI LIAT GSEV TPFP GITI DEDT IVSS TGAL SLKK VPEK MVVI GAGV IGVE LGSV WQRL GADV TAVE FLGH VGGV GIDM EISK NFQR ILQK QGFK FKLN TKVT GATK KSDG KIDV SIEA ASGG K |
遺伝子ID: | 1738 |
Uniprot: | P09622 |
セルラーロケーション: | Mitochondrion matrix |
計算された分子量: | 43kDa/49kDa/54kDa |
観察された分子量: | 55kDa |
同義語: | DLD, DLDD, DLDH, E3, GCSL, LAD, PHE3 |
バックグラウンド: | 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. |
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 |