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Mouse Anti-ETFDH Recombinant Antibody (CBFYE-1329) (CBMAB-E1937-FY)

This product is mouse antibody that recognizes ETFDH. The antibody CBFYE-1329 can be used for immunoassay techniques such as: WB, IHC-P, FC, IF, ELISA.
See all ETFDH antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Cattle, Human
Clone
CBFYE-1329
Antibody Isotype
IgG1
Application
WB, IHC-P, FC, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Cattle, Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Electron Transfer Flavoprotein Dehydrogenase
Introduction
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.
Entrez Gene ID
Human2110
Mouse66841
Rat295143
Cattle768074
UniProt ID
HumanQ16134
MouseQ921G7
RatQ6UPE1
CattleQ2KIG0
Alternative Names
Electron Transfer Flavoprotein Dehydrogenase; Electron-Transferring-Flavoprotein Dehydrogenase; ETF-Ubiquinone Oxidoreductase; ETF Dehydrogenase; EC 1.5.5.1; ETF-QO; Electron Transfer Flavoprotein-Ubiquinone Oxidoreductase, Mitochondrial; ETFQO; MADD
Research Area
Accepts electrons from ETF and reduces ubiquinone.
Biological Process
Electron transport chain Source: UniProtKB
Fatty acid beta-oxidation using acyl-CoA dehydrogenase Source: BHF-UCL
Respiratory electron transport chain Source: Reactome
Response to oxidative stress Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Glutaric aciduria 2C (GA2C):
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.

Ali, A., Dhahouri, N. A., Almesmari, F. S. A., Fathalla, W. M., & Jasmi, F. A. (2021). Characterization of ETFDH and PHGDH mutations in a patient with mild glutaric aciduria type II and serine deficiency. Genes, 12(5), 703.

Hu, G., Zeng, J., Wang, C., Zhou, W., Jia, Z., Yang, J., & Zheng, B. (2020). A Synonymous Variant c. 579A> G in the ETFDH gene caused exon skipping in a patient with late-onset multiple acyl-coa dehydrogenase deficiency: a case report. Frontiers in Pediatrics, 8, 118.

Ou, M., Zhu, L., Zhang, Y., Zhang, Y., Zhou, J., Zhang, Y., ... & Wang, W. (2020). A novel electron transfer flavoprotein dehydrogenase (ETFDH) gene mutation identified in a newborn with glutaric acidemia type II: a case report of a Chinese family. BMC Medical Genetics, 21(1), 1-7.

Ding, M., Liu, R., Qiubo, L., Zhang, Y., & Kong, Q. (2020). Neonatal-onset multiple acyl-CoA dehydrogenase deficiency (MADD) in the ETFDH gene: A case report and a literature review. Medicine, 99(37).

Missaglia, S., Pegoraro, V., Marozzo, R., Tavian, D., & Angelini, C. (2020). Correlation between ETFDH mutations and dysregulation of serum myomiRs in MADD patients. European Journal of Translational Myology, 30(1).

Chen, W., Zhang, Y., Ni, Y., Cai, S., Zheng, X., Mastaglia, F. L., & Wu, J. (2019). Late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (MADD): case reports and epidemiology of ETFDH gene mutations. BMC neurology, 19(1), 1-7.

Wu, Y., Zhang, X., Shen, R., Huang, J., Lu, X., Zheng, G., & Chen, X. (2019). Expression and significance of ETFDH in hepatocellular carcinoma. Pathology-Research and Practice, 215(12), 152702.

Missaglia, S., Tavian, D., Moro, L., & Angelini, C. (2018). Characterization of two ETFDH mutations in a novel case of riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency. Lipids in health and disease, 17(1), 1-8.

Fan, X., Xie, B., Zou, J., Luo, J., Qin, Z., D'Gama, A. M., ... & Shen, Y. (2018). Novel ETFDH mutations in four cases of riboflavin responsive multiple acyl-CoA dehydrogenase deficiency. Molecular Genetics and Metabolism Reports, 16, 15-19.

Xu, J., Li, D., Lv, J., Xu, X., Wen, B., Lin, P., ... & Yan, C. (2018). ETFDH Mutations and Flavin Adenine Dinucleotide Homeostasis Disturbance Are Essential for Developing Riboflavin‐Responsive Multiple Acyl–Coenzyme A Dehydrogenation Deficiency. Annals of Neurology, 84(5), 659-673.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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