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Mouse Anti-NDUFS4 Recombinant Antibody (A-7) (CBMAB-N1688-WJ)

This product is a Mouse antibody that recognizes NDUFS4. The antibody A-7 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all NDUFS4 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
A-7
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Human
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!]

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) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
Introduction
This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015]
Entrez Gene ID
4724
UniProt ID
O43181
Alternative Names
NADH:Ubiquinone Oxidoreductase Subunit S4; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase); NADH-Ubiquinone Oxidoreductase 18 KDa Subunit; Complex I 18kDa Subunit; Complex I-AQDQ; CI-18 KDa; CI-AQDQ;
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Biological Process
Aerobic respiration Source: ComplexPortal
Brain development Source: UniProtKB
Cellular respiration Source: UniProtKB
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Positive regulation of fibroblast proliferation Source: UniProtKB
Reactive oxygen species metabolic process Source: UniProtKB
Regulation of protein phosphorylation Source: MGI
Response to cAMP Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Note: The interaction with BCAP31 mediates mitochondria localization.
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 1 (MC1DN1):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.

van Rensburg, D. J., Lindeque, Z., Harvey, B. H., & Steyn, S. F. (2024). Ndufs4 KO mice: A model to study comorbid mood disorders associated with mitochondrial dysfunction. Pharmacology Biochemistry and Behavior, 234, 173689.

Hemmi, T., Suzuki, J., Kagawa, Y., Honkura, Y., Ikeda, R., Hashimoto, K., ... & Katori, Y. (2023). Effects of Ndufs4 deletion on hearing after various acoustic exposures. The Tohoku Journal of Experimental Medicine, 260(3), 181-191.

Van De Wal, M. A., Adjobo-Hermans, M. J., Keijer, J., Schirris, T. J., Homberg, J. R., Wieckowski, M. R., ... & Koopman, W. J. (2022). Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention. Brain, 145(1), 45-63.

Miller, H. C., Louw, R., Mereis, M., Venter, G., Boshoff, J. D., Mienie, L., ... & van der Westhuizen, F. H. (2021). Metallothionein 1 overexpression does not protect against mitochondrial disease pathology in Ndufs4 knockout mice. Molecular Neurobiology, 58, 243-262.

Kahlhöfer, F., Gansen, M., & Zickermann, V. (2021). Accessory subunits of the matrix arm of mitochondrial complex I with a focus on subunit NDUFS4 and its role in complex I function and assembly. Life, 11(5), 455.

Shil, S. K., Kagawa, Y., Umaru, B. A., Nanto-Hara, F., Miyazaki, H., Yamamoto, Y., ... & Owada, Y. (2021). Ndufs4 ablation decreases synaptophysin expression in hippocampus. Scientific reports, 11(1), 10969.

Adjobo-Hermans, M. J., De Haas, R., Willems, P. H., Wojtala, A., van Emst-de Vries, S. E., Wagenaars, J. A., ... & Koopman, W. J. (2020). NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4−/− mice and Leigh syndrome patients: A stabilizing role for NDUFAF2. Biochimica et Biophysica Acta (BBA)-Bioenergetics, 1861(8), 148213.

González-Quintana, A., Trujillo-Tiebas, M. J., Fernández-Perrone, A. L., Blázquez, A., Lucia, A., Morán, M., ... & Martín, M. A. (2020). Uniparental isodisomy as a cause of mitochondrial complex I respiratory chain disorder due to a novel splicing NDUFS4 mutation. Molecular Genetics and Metabolism, 131(3), 341-348.

Johnson, S. C., Kayser, E. B., Bornstein, R., Stokes, J., Bitto, A., Park, K. Y., ... & Morgan, P. G. (2020). Regional metabolic signatures in the Ndufs4 (KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease. Molecular genetics and metabolism, 130(2), 118-132.

Terburgh, K., Lindeque, Z., Mason, S., Van der Westhuizen, F., & Louw, R. (2019). Metabolomics of Ndufs4−/− skeletal muscle: Adaptive mechanisms converge at the ubiquinone-cycle. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1865(1), 98-106.

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

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