Rabbit Anti-NDUFS3 Recombinant Antibody (CBWJN-0967) (CBMAB-N0216-WJ)

Name: Rabbit Anti-NDUFS3 Recombinant Antibody (CBWJN-0967)
SKU: CBMAB-N0216-WJ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

CBWJN-0967

Application

WB, IP, IHC-P, ICC

Specificity

Mouse, Rat, Human

Antibody Isotype

IgG

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.

More Infomation

Target

Full Name

NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)

Introduction

This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.[provided by RefSeq, Apr 2009]

Entrez Gene ID

Human	4722
Mouse	68349
Rat	295923

UniProt ID

Human	O75489
Mouse	Q9DCT2
Rat	D3ZG43

Alternative Names

NADH:Ubiquinone Oxidoreductase Core Subunit S3; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 3, 30kDa (NADH-Coenzyme Q Reductase); NADH Dehydrogenase [Ubiquinone] Iron-Sulfur Protein 3, Mitochondrial; NADH-Ubiquinone Oxidoreductase 30 KDa Subunit; Complex I 30kDa Subunit; Complex I-30kD; CI-30kD;

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:14729820, PubMed:30140060).

Essential for the catalytic activity and assembly of complex I (PubMed:14729820, PubMed:24028823, PubMed:30140060).

Biological Process

Aerobic respiration Source: ComplexPortal
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Negative regulation of cell growth Source: UniProtKB
Negative regulation of intrinsic apoptotic signaling pathway Source: UniProtKB
Reactive oxygen species metabolic process Source: UniProtKB
Substantia nigra development Source: UniProtKB

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Mitochondrial complex I deficiency, nuclear type 8 (MC1DN8):
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. MC1DN8 transmission pattern is consistent with autosomal recessive inheritance.

Ma, Y. H., Yang, Y., Li, J. H., Yao, B. C., Chen, Q. L., Wang, L. Q., ... & Guo, S. Z. (2023). NDUFB11 and NDUFS3 regulate arterial atherosclerosis and venous thrombosis: Potential markers of atherosclerosis and venous thrombosis. Medicine, 102(46), e36133.

Kurelac, I., Cavina, B., Sollazzo, M., Miglietta, S., Fornasa, A., De Luise, M., ... & Gasparre, G. (2022). NDUFS3 knockout cancer cells and molecular docking reveal specificity and mode of action of anti-cancer respiratory complex I inhibitors. Open Biology, 12(11), 220198.

D’Angelo, L., Astro, E., De Luise, M., Kurelac, I., Umesh-Ganesh, N., Ding, S., ... & Iommarini, L. (2021). NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate. Cell Reports, 35(3).

Samanta, D., Veerapandiyan, A., Burrow, T. A., & Gokden, M. (2021). Mitochondrial ultrastructural defects in NDUFS3-related disorder. Journal of Pediatric Neurosciences, 16(4), 299.

D’Angelo, L., Astro, E., De Luise, M., Kurelac, I., Umesh-Ganesh, N., Ding, S., ... & Iommarini, L. (2020). Biogenesis of NDUFS3-less complex I indicates TMEM126A/OPA7 as an assembly factor of the ND4-module. bioRxiv, 2020-10.

Chanthammachat, P., & Dharmasaroja, P. (2019). Metformin restores the mitochondrial membrane potentials in association with a reduction in TIMM23 and NDUFS3 in MPP+-induced neurotoxicity in SH-SY5Y cells. EXCLI journal, 18, 812.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

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

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