Anti-ETFA Antibody (CAB19937)[KO Validated]
- SKU:
- CAB19937
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-ETFA Antibody [KO Validated] |
抗体コード: | CAB19937 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant protein of human ETFA. |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | 293T |
免疫原: | Recombinant protein of human ETFA. |
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: | Email for sequence |
遺伝子ID: | 2108 |
Uniprot: | P13804 |
セルラーロケーション: | Mitochondrion matrix |
計算された分子量: | 30kDa/35kDa |
観察された分子量: | 30KDa |
同義語: | ETFA, EMA, GA2, MADD |
バックグラウンド: | ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. |
UniProt Protein Function: | ETFA: The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl- CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Defects in ETFA are the cause of glutaric aciduria type 2A (GA2A); also known as glutaricaciduria IIA. GA2A is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF alpha-subunit/FixB family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 15q23-q25 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; oxidoreductase activity Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis Disease: Multiple Acyl-coa Dehydrogenase Deficiency |
NCBI Summary: | ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | P13804 |
NCBI GenInfo Identifier: | 119636 |
NCBI Gene ID: | 2108 |
NCBI Accession: | P13804.1 |
UniProt Secondary Accession: | P13804,Q53XN3, B4DT43, |
UniProt Related Accession: | P13804 |
Molecular Weight: | 30,026 Da |
NCBI Full Name: | Electron transfer flavoprotein subunit alpha, mitochondrial |
NCBI Synonym Full Names: | electron transfer flavoprotein alpha subunit |
NCBI Official Symbol: | ETFA |
NCBI Official Synonym Symbols: | EMA; GA2; MADD |
NCBI Protein Information: | electron transfer flavoprotein subunit alpha, mitochondrial |
UniProt Protein Name: | Electron transfer flavoprotein subunit alpha, mitochondrial |
Protein Family: | Electron transfer flavoprotein |
UniProt Gene Name: | ETFA |
UniProt Entry Name: | ETFA_HUMAN |