Rabbit Anti-NDUFS6 Recombinant Antibody (CBWJN-1280) (CBMAB-N1694-WJ)

Name: Rabbit Anti-NDUFS6 Recombinant Antibody (CBWJN-1280)
SKU: CBMAB-N1694-WJ
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Rabbit

Specificity

Mouse, Rat, Human

Clone

CBWJN-1280

Antibody Isotype

IgG

Application

WB, IHC-P, FC, ICC

Basic Information

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!]

Concentration

0.465 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 Subunit S6

Introduction

This gene encodes a subunit of the NADH:ubiquinone oxidoreductase (complex I), which is the first enzyme complex in the electron transport chain of mitochondria. This complex functions in the transfer of electrons from NADH to the respiratory chain. The subunit encoded by this gene is one of seven subunits in the iron-sulfur protein fraction. Mutations in this gene cause mitochondrial complex I deficiency, a disease that causes a wide variety of clinical disorders, including neonatal disease and adult-onset neurodegenerative disorders.[provided by RefSeq, Oct 2009]

Entrez Gene ID

Human	4726
Mouse	407785
Rat	29478

UniProt ID

Human	O75380
Mouse	P52503
Rat	P52504

Alternative Names

NADH:Ubiquinone Oxidoreductase Subunit S6; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 6, 13kDa (NADH-Coenzyme Q Reductase); NADH Dehydrogenase [Ubiquinone] Iron-Sulfur Protein 6, Mitochondrial; NADH-Ubiquinone Oxidoreductase 13 KDa-A Subunit; Complex I 13kDa Subunit A; CI-13kD-A; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 6 (13kD) (NADH-Coenzyme Q Reductase);

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
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: GO_Central

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Mitochondrial complex I deficiency, nuclear type 9 (MC1DN9):
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. MC1DN9 transmission pattern is consistent with autosomal recessive inheritance.

More Infomation

References

Gangfuß, A., Ferreira, T., Hentschel, A., Marina, A. D., Kölbel, H., Sickmann, A., ... & Roos, A. (2024). A Homozygous NDUFS6 Variant Associated with Neuropathy and Optic Atrophy. Journal of Neuromuscular Diseases, (Preprint), 1-7.

Jia, Y., Yu, X., Liu, R., Shi, L., Jin, H., Yang, D., ... & Kong, G. (2023). PRMT1 methylation of WTAP promotes multiple myeloma tumorigenesis by activating oxidative phosphorylation via m6A modification of NDUFS6. Cell Death & Disease, 14(8), 512.

Li, Y., Zhang, Y., Jiang, G., Wang, Y., He, C., Zhao, X., ... & Li, L. (2022). Case report: novel mutations of NDUFS6 and NHLRC2 genes potentially cause the quick postnatal death of a Chinese Hani minority neonate with mitochondrial complex I deficiency and FINCA syndrome. Medicine, 101(27).

Zhang, Y., Guo, L., Han, S., Chen, L., Li, C., Zhang, Z., ... & Lian, Q. (2020). Adult mesenchymal stem cell ageing interplays with depressed mitochondrial Ndufs6. Cell death & disease, 11(12), 1075.

Rouzier, C., Chaussenot, A., Fragaki, K., Serre, V., Ait-El-Mkadem, S., Richelme, C., ... & Bannwarth, S. (2019). NDUFS6 related Leigh syndrome: a case report and review of the literature. Journal of human genetics, 64(7), 637-645.

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Protocols

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)
Proteogenomics
Other Protocols

Associated Products

Isotype control

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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Documents

Datasheet
SDS
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