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Mouse Anti-NDUFS2 Recombinant Antibody (1D5) (CBMAB-N1676-WJ)

This product is a Mouse antibody that recognizes NDUFS2. The antibody 1D5 can be used for immunoassay techniques such as: WB, IHC .
See all NDUFS2 antibodies
Published Data
Fig.1 Western blot for the mitochondrial complex I components in CI and CII PCPGs confirmed overexpression of complex I.
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Fig.2 Immunohistochemistry of mitochondrial complex I components in cluster I (CI) neuroendocrine tumor specimens showed upregulation of complex I subunits NDUFS2.
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Summary

Host Animal
Mouse
Specificity
Human
Clone
1D5
Antibody Isotype
IgG1
Application
WB, IHC

Basic Information

Specificity
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
Buffer
PBS, pH 7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
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
NADH:Ubiquinone Oxidoreductase Core Subunit S2
Introduction
The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]
Entrez Gene ID
4720
UniProt ID
O75306
Alternative Names
NADH:Ubiquinone Oxidoreductase Core Subunit S2; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 2, 49kDa (NADH-Coenzyme Q Reductase); NADH Dehydrogenase [Ubiquinone] Iron-Sulfur Protein 2, Mitochondrial; NADH-Ubiquinone Oxidoreductase 49 KDa Subunit; Complex I 49kDa Subunit; Complex I-49kD; CI-49kD; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 2 (49kD) (NADH-Coenzyme Q Reductase);
Function
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:30922174, PubMed:22036843).

Essential for the catalytic activity of complex I (PubMed:30922174, PubMed:22036843).

Essential for the assembly of complex I (By similarity).

Redox-sensitive, critical component of the oxygen-sensing pathway in the pulmonary vasculature which plays a key role in acute pulmonary oxygen-sensing and hypoxic pulmonary vasoconstriction (PubMed:30922174).

Plays an important role in carotid body sensing of hypoxia (By similarity).

Essential for glia-like neural stem and progenitor cell proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation (By similarity).
Biological Process
Aerobic respiration Source: ComplexPortal
Cellular response to oxygen levels Source: UniProtKB
Gliogenesis Source: UniProtKB
Mitochondrial ATP synthesis coupled electron transport Source: CAFA
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Neural precursor cell proliferation Source: UniProtKB
Neurogenesis Source: UniProtKB
Response to oxidative stress Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 6 (MC1DN6):
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. MC1DN6 transmission pattern is consistent with autosomal recessive inheritance.
PTM
Dimethylation at Arg-118 by NDUFAF7 takes place after NDUFS2 assembles into the complex I, leading to stabilize the early intermediate complex (PubMed:24089531, PubMed:24838397).

McElroy, G. S., Chakrabarty, R. P., D’Alessandro, K. B., Hu, Y. S., Vasan, K., Tan, J., ... & Chandel, N. S. (2022). Reduced expression of mitochondrial complex I subunit Ndufs2 does not impact healthspan in mice. Scientific reports, 12(1), 5196.

Bandara, A. B., Drake, J. C., James, C. C., Smyth, J. W., & Brown, D. A. (2021). Complex I protein NDUFS2 is vital for growth, ROS generation, membrane integrity, apoptosis, and mitochondrial energetics. Mitochondrion, 58, 160-168.

Yang, Y., Yang, X., Lin, Y., Yang, G., & Li, L. (2020). LASS2 regulates hepatocyte steatosis by interacting with NDUFS2/OXPHOS related proteins. Biochemical and Biophysical Research Communications, 526(4), 871-879.

Liu, L., Qi, L., Knifley, T., Piecoro, D. W., Rychahou, P., Liu, J., ... & Chen, M. (2019). S100A4 alters mitochondrial metabolism to promote invasion and metastasis of non-small cell lung cancer cells through upregulation of NDUFS2. Cancer Research, 79(13_Supplement), 1119-1119.

Wolin, M. S., Alruwaili, N., & Kandhi, S. (2019). Studies on hypoxic pulmonary vasoconstriction detect a novel role for the mitochondrial complex I subunit Ndufs2 in controlling peroxide generation for oxygen-sensing. Circulation research, 124(12), 1683-1685.

Liu, L., Qi, L., Knifley, T., Piecoro, D. W., Rychahou, P., Liu, J., ... & Chen, M. (2019). S100A4 alters metabolism and promotes invasion of lung cancer cells by up-regulating mitochondrial complex I protein NDUFS2. Journal of Biological Chemistry, 294(18), 7516-7527.

Dunham-Snary, K. J., Wu, D., Potus, F., Sykes, E. A., Mewburn, J. D., Charles, R. L., ... & Archer, S. L. (2019). Ndufs2, a core subunit of mitochondrial complex I, is essential for acute oxygen-sensing and hypoxic pulmonary vasoconstriction. Circulation research, 124(12), 1727-1746.

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

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