Anti-ETFDH Antibody (CAB6585)
- SKU:
- CAB6585
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-ETFDH Antibody |
| 抗体コード: | CAB6585 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 318-617 of human ETFDH (NP_004444.2). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | Mouse liver, Mouse heart, Mouse kidney, Rat liver, Rat heart |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 318-617 of human ETFDH (NP_004444.2). |
| 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: | LVAL GLVV GLDY QNPY LSPF REFQ RWKH HPSI RPTL EGGK RIAY GARA LNEG GFQS IPKL TFPG GLLI GCSP GFMN VPKI KGTH TAMK SGIL AAES IFNQ LTSE NLQS KTIG LHVT EYED NLKN SWVW KELY SVRN IRPS CHGV LGVY GGMI YTGI FYWI LRGM EPWT LKHK GSDF ERLK PAKD CTPI EYPK PDGQ ISFD LLSS VALS GTNH EHDQ PAHL TLRD DSIP VNRN LSIY DGPE QRFC PAGV YEFV PVEQ GDGF RLQI NAQN CVHC KTCD IKDP SQNI NWVV PEGG GGPA YNGM |
| 遺伝子ID: | 2110 |
| Uniprot: | Q16134 |
| セルラーロケーション: | Mitochondrion inner membrane |
| 計算された分子量: | 62kDa/68kDa |
| 観察された分子量: | 65kDa |
| 同義語: | ETFDH, ETFQO, MADD |
| バックグラウンド: | This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. |
| UniProt Protein Function: | ETFDH: Accepts electrons from ETF and reduces ubiquinone. Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C). GA2C 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-QO/FixC family. |
| UniProt Protein Details: | Protein type:Membrane protein, integral; Mitochondrial; Oxidoreductase; EC 1.5.5.1 Chromosomal Location of Human Ortholog: 4q32-q35 Cellular Component: mitochondrial matrix; mitochondrial membrane; integral to mitochondrial inner membrane Molecular Function:ubiquinone binding; FAD binding; electron carrier activity; metal ion binding; 4 iron, 4 sulfur cluster binding; oxidoreductase activity, oxidizing metal ions with flavin as acceptor; oxidoreductase activity; quinone binding; electron-transferring-flavoprotein dehydrogenase activity Biological Process: cellular metabolic process; response to oxidative stress; fatty acid beta-oxidation using acyl-CoA dehydrogenase Disease: Multiple Acyl-coa Dehydrogenase Deficiency |
| NCBI Summary: | This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. [provided by RefSeq, Aug 2013] |
| UniProt Code: | Q16134 |
| NCBI GenInfo Identifier: | 292495008 |
| NCBI Gene ID: | 2110 |
| NCBI Accession: | Q16134.2 |
| UniProt Secondary Accession: | Q16134,Q7Z347, B4E3R9, J3KND9, |
| UniProt Related Accession: | Q16134,AAB24227 |
| Molecular Weight: | 68,495 Da |
| NCBI Full Name: | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial |
| NCBI Synonym Full Names: | electron-transferring-flavoprotein dehydrogenase |
| NCBI Official Symbol: | ETFDH |
| NCBI Official Synonym Symbols: | MADD; ETFQO |
| NCBI Protein Information: | electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; ETF-QO; ETF dehydrogenase; ETF-ubiquinone oxidoreductase |
| UniProt Protein Name: | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial |
| UniProt Synonym Protein Names: | Electron-transferring-flavoprotein dehydrogenase |
| UniProt Gene Name: | ETFDH |
| UniProt Entry Name: | ETFD_HUMAN |
 | Western blot analysis of extracts of various cell lines, using ETFDH antibody (CAB6585) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 3s. |
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