ETFDH Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q16134 |
| Other Accession | Q16134 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 68495 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human ETFDH |
| Epitope Specificity | 41-150/617 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the ETF-QO/fixC family. Contains 1 4Fe-4S ferredoxin-type domain. |
| SUBUNIT | Monomer. |
| DISEASE | Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C) [MIM:231680]. 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia. [provided by RefSeq, Jul 2008]. |
| Gene ID | 2110 |
|---|---|
| Other Names | ETFQO; MADD; 0610010I20Rik; ETFD_BOVIN; ETFDH; ETF-QO; ETF-ubiquinone oxidoreductase; Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase); 1.5.5.1; ETFD_HUMAN; ETFD_MOUSE; ETFD_RAT; electron transfer flavoprotein dehydrogenase; electron-transferring-flavoprotein dehydrogenase |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | ETFDH (HGNC:3483) |
|---|---|
| Function | Links fatty acid beta-oxidation and amino acid catabolism to the respiratory chain by transferring electrons from the electron transfer flavoprotein (ETF) to ubiquinone. |
| Cellular Location | Mitochondrion inner membrane. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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