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Rabbit Anti-NDUFC2 Recombinant Antibody (CBWJN-1273) (CBMAB-N1673-WJ)

This product is a Rabbit antibody that recognizes NDUFC2. The antibody CBWJN-1273 can be used for immunoassay techniques such as: WB, IHC-P, FC.
See all NDUFC2 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
CBWJN-1273
Antibody Isotype
IgG
Application
WB, IHC-P, FC

Basic Information

Specificity
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
0.182 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 C2
Introduction
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.
Entrez Gene ID
UniProt ID
Alternative Names
NADH:Ubiquinone Oxidoreductase Subunit C2; NADH Dehydrogenase (Ubiquinone) 1, Subcomplex Unknown, 2, 14.5kDa; NADH-Ubiquinone Oxidoreductase Subunit B14.5b; Human Lung Cancer Oncogene 1 Protein; Complex I Subunit B14.5b; Complex I-B14.5b; CI-B14.5b;
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis but required for the complex assembly. 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: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 36 (MC1DN36):
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. MC1DN36 is characterized by global developmental delay, hypotonia, and failure to thrive apparent from infancy or early childhood. Affected individuals usually do not acquire ambulation, show progressive spasticity, and have impaired intellectual development with absent speech. MC1DN36 transmission pattern is consistent with autosomal recessive inheritance.
Topology
Helical: 56-75

Chen, J., Liu, X., Bi, R., Liu, P., & Gong, W. (2023). NDUFC2 deficiency exacerbates endothelial mesenchymal transformation during ischemia-reperfusion via NLRP3. NeuroReport, 34(13), 670-676.

Gallo, G., Forte, M., Cotugno, M., Marchitti, S., Stanzione, R., Tocci, G., ... & Rubattu, S. (2023). Polymorphic variants at NDUFC2, encoding a mitochondrial complex I subunit, associate with cardiac hypertrophy in human hypertension. Molecular Medicine, 29(1), 107.

Chi, X., Wu, Y., Zhang, C., Duan, S., Li, Y., Bajpai, A. K., ... & Xu, Z. (2023). TEAD1, MYO7A and NDUFC2 are novel functional genes associated with glucose metabolism in BXD recombinant inbred population.

Gallo, G., Migliarino, S., Cotugno, M., Stanzione, R., Burocchi, S., Bianchi, F., ... & Rubattu, S. (2022). Impact of a NDUFC2 Variant on the Occurrence of Acute Coronary Syndromes. Frontiers in Cardiovascular Medicine, 9, 921244.

Alahmad, A., Nasca, A., Heidler, J., Thompson, K., Oláhová, M., Legati, A., ... & Taylor, R. W. (2020). Bi‐allelic pathogenic variants in NDUFC2 cause early‐onset Leigh syndrome and stalled biogenesis of complex I. EMBO molecular medicine, 12(11), e12619.

Huang, J., Zheng, Q., Wang, S., Wei, X., Li, F., & Ma, Y. (2019). High-throughput RNA sequencing reveals NDUFC2-AS lncRNA promotes adipogenic differentiation in chinese buffalo (Bubalus bubalis L.). Genes, 10(9), 689.

Raffa, S., Chin, X. L. D., Stanzione, R., Forte, M., Bianchi, F., Cotugno, M., ... & Rubattu, S. (2019). The reduction of NDUFC2 expression is associated with mitochondrial impairment in circulating mononuclear cells of patients with acute coronary syndrome. International journal of cardiology, 286, 127-133.

Madonna, R., Vitulli, P., Pierdomenico, S., & De Caterina, R. (2019). Deficiency of NDUFC2: Cause or bystander in acute coronary syndromes?. International Journal of Cardiology, 286, 134-135.

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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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