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Mouse Anti-NDUFA8 Recombinant Antibody (2E10) (CBMAB-N1618-WJ)

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Datasheet
SDS
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Basic Information

Host Animal
Mouse
Clone
2E10
Application
ELISA, IHC, WB
Specificity
Human
Antibody Isotype
IgG2a, κ
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 7.2
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) 1 alpha subcomplex, 8, 19kDa
Introduction
The protein encoded by this gene belongs to the complex I 19 kDa subunit family. Mammalian complex I is composed of 45 different subunits. This protein has NADH dehydrogenase activity and oxidoreductase activity. It plays an important role in transfering electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2015]
Entrez Gene ID
4702
UniProt ID
P51970
Alternative Names
NADH:Ubiquinone Oxidoreductase Subunit A8; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex, 8, 19kDa; NADH-Ubiquinone Oxidoreductase 19 KDa Subunit; Complex I-19kD; Complex I-PGIV; CI-19KD; CI-PGIV;
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis (PubMed:27626371, PubMed:32385911, PubMed:33153867).

Complex I functions in the transfer of electrons from NADH to the respiratory chain (PubMed:27626371).

The immediate electron acceptor for the enzyme is believed to be ubiquinone (PubMed:27626371).
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
Mitochondrion inner membrane
Mitochondrion intermembrane space
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 37 (MC1DN37):
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. MC1DN37 features include developmental delay, cerebral atrophy, epilepsy, growth retardation, congenital myopathy with disproportion of fibers, and severely decreased activity of complex I. MC1DN37 transmission pattern is consistent with autosomal recessive inheritance.
PTM
May contain intrachain disulfide bonds, as evidenced by its electrophoretic mobility under reducing vs non-reducing conditions.

Xiang, H., Tang, H., He, Q., Sun, J., Yang, Y., Kong, L., & Wang, Y. (2024). NDUFA8 is transcriptionally regulated by EP300/H3K27ac and promotes mitochondrial respiration to support proliferation and inhibit apoptosis in cervical cancer. Biochemical and Biophysical Research Communications, 693, 149374.

Chen, J., Wang, M. K., Xie, Q. X., Bing, X. L., Li, T. P., & Hong, X. Y. (2023). NDUFA8 potentially rescues Wolbachia‐induced cytoplasmic incompatibility in Laodelphax striatellus. Insect Science.

Yatsuka, Y., Kishita, Y., Formosa, L. E., Shimura, M., Nozaki, F., Fujii, T., ... & Okazaki, Y. (2020). A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency. Clinical Genetics, 98(2), 155-165.

Tort, F., Barredo, E., Parthasarathy, R., Ugarteburu, O., Ferrer-Cortes, X., Garcia-Villoria, J., ... & Ribes, A. (2020). Biallelic mutations in NDUFA8 cause complex I deficiency in two siblings with favorable clinical evolution. Molecular Genetics and Metabolism, 131(3), 349-357.

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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)
Proteogenomics
Other Protocols

Related Products

Rabbit Anti-NDUFA8 Recombinant Antibody (CBWJN-1255) (CAT#: CBMAB-N1619-WJ)

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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