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Mouse Anti-NDUFA4 Recombinant Antibody (2G7) (CBMAB-N1603-WJ)

This product is a Mouse antibody that recognizes NDUFA4. The antibody 2G7 can be used for immunoassay techniques such as: ELISA, WB.
See all NDUFA4 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2G7
Antibody Isotype
IgG, λ
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG, λ
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
Buffer
PBS, pH 7.4
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
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa
Introduction
The protein encoded by this gene belongs to the complex I 9kDa subunit family. Mammalian complex I of mitochondrial respiratory chain is composed of 45 different subunits. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. [provided by RefSeq, Jul 2008]
Entrez Gene ID
4697
UniProt ID
O00483
Alternative Names
NDUFA4, Mitochondrial Complex Associated; NADH-Ubiquinone Oxidoreductase MLRQ Subunit; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex, 4, 9kDa; Complex I 9kDa Subunit; Complex I-MLRQ; CI-MLRQ;
Function
Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix (PubMed:22902835).

NDUFA4 is required for complex IV maintenance (PubMed:22902835).
Biological Process
Cellular respiration Source: ComplexPortal
Mitochondrial electron transport, cytochrome c to oxygen Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Positive regulation of cytochrome-c oxidase activity Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex IV deficiency, nuclear type 21 (MC4DN21):
An autosomal recessive mitochondrial disorder with onset in infancy. MC4DN21 is characterized by congenital lactic acidosis, encephalopathy, global developmental delay, delayed speech, motor dysfunction, dystonia, and spasticity. Ataxia, peripheral neuropathy, and seizures may also occur. Patient tissues show variably decreased levels and activity of mitochondrial respiratory complex IV.
Topology
Mitochondrial matrix: 1-14
Helical: 15-37
Mitochondrial intermembrane: 38-81

Fu, F., Chen, C., Du, K., Li, L. S., Li, R., Lei, T. Y., ... & Liao, C. (2023). Ndufa4 Regulates the Proliferation and Apoptosis of Neurons via miR-145a-5p/Homer1/Ccnd2. Molecular Neurobiology, 60(6), 2986-3003.

Zhu, J., Xiang, X., Hu, X., Li, C., Song, Z., & Dong, Z. (2023). miR-147 represses NDUFA4, inducing mitochondrial dysfunction and tubular damage in cold storage kidney transplantation. Journal of the American Society of Nephrology, 34(8), 1381-1397.

Qin, S., You, P., Yu, H., & Su, B. (2023). REEP1 Preserves Motor Function in SOD1G93A Mice by Improving Mitochondrial Function via Interaction with NDUFA4. Neuroscience Bulletin, 39(6), 929-946.

Wang, Z., Tao, E., Chen, Y., Wang, Q., Liu, M., Wei, L., ... & Zhong, C. (2023). NDUFA4 promotes the progression of head and neck paraganglioma by inhibiting ferroptosis. Biochemistry and Cell Biology, 101(6), 523-530.

Xu, W., Lai, Y., Pan, Y., Tan, M., Ma, Y., Sheng, H., & Wang, J. (2022). m6A RNA methylation-mediated NDUFA4 promotes cell proliferation and metabolism in gastric cancer. Cell Death & Disease, 13(8), 715.

Zhang, Y., Ge, M., Chen, Y., Yang, Y., Chen, W., Wu, D., ... & Wu, X. (2022). NDUFA4 promotes cell proliferation by enhancing oxidative phosphorylation in pancreatic adenocarcinoma. Journal of Bioenergetics and Biomembranes, 54(5-6), 283-291.

Han, Y., Tan, L., Zhou, T., Yang, L., Carrau, L., Lacko, L. A., ... & Chen, S. (2022). A human iPSC-array-based GWAS identifies a virus susceptibility locus in the NDUFA4 gene and functional variants. Cell Stem Cell, 29(10), 1475-1490.

Chen, D., Hou, Y., & Cai, X. (2021). MiR-210-3p enhances cardiomyocyte apoptosis and mitochondrial dysfunction by targeting the NDUFA4 gene in sepsis-induced myocardial dysfunction. International Heart Journal, 62(3), 636-646.

Tian, F., Tang, P., Sun, Z., Zhang, R., Zhu, D., He, J., ... & Shen, J. (2020). miR-210 in exosomes derived from macrophages under high glucose promotes mouse diabetic obesity pathogenesis by suppressing NDUFA4 expression. Journal of Diabetes Research, 2020.

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

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