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Mouse Anti-MT-ND1 Recombinant Antibody (3H3) (CBMAB-A5823-LY)

The product is antibody recognizes ND1. The antibody 3H3 immunoassay techniques such as: WB, ELISA.
See all MT-ND1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3H3
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
ND1 (YP_003024026, 21 a.a. ~ 71 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
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
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
MITOCHONDRIALLY ENCODED NADH:UBIQUINONE OXIDOREDUCTASE CORE SUBUNIT 1
Entrez Gene ID
UniProt ID
Alternative Names
MTND1
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:1959619).

Essential for the catalytic activity and assembly of complex I (PubMed:26929434, PubMed:1959619).
Biological Process
Aerobic respiration Source: GO_Central
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
Leber hereditary optic neuropathy (LHON):
A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS):
Genetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.
Alzheimer disease mitochondrial (AD-MT):
Alzheimer disease is a neurodegenerative disorder characterized by progressive dementia, loss of cognitive abilities, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major constituents of these plaques are neurotoxic amyloid-beta protein 40 and amyloid-beta protein 42, that are produced by the proteolysis of the transmembrane APP protein. The cytotoxic C-terminal fragments (CTFs) and the caspase-cleaved products, such as C31, are also implicated in neuronal death.
Diabetes mellitus, non-insulin-dependent (NIDDM):
A multifactorial disorder of glucose homeostasis caused by a lack of sensitivity to the body's own insulin. Affected individuals usually have an obese body habitus and manifestations of a metabolic syndrome characterized by diabetes, insulin resistance, hypertension and hypertriglyceridemia. The disease results in long-term complications that affect the eyes, kidneys, nerves, and blood vessels.

Xu, Y. C., Su, J., Zhou, J. J., Yuan, Q., & Han, J. S. (2023). Roles of MT-ND1 in Cancer. Current Medical Science, 43(5), 869-878.

Lou, X., Zhou, Y., Liu, Z., Xie, Y., Zhang, L., Zhao, S., ... & Lyu, J. (2023). De novo frameshift variant in MT-ND1 causes a mitochondrial complex I deficiency associated with MELAS syndrome. Gene, 860, 147229.

Xu, M., Kopajtich, R., Elstner, M., Li, H., Liu, Z., Wang, J., ... & Fang, F. (2022). Identification of a novel m. 3955G> A variant in MT-ND1 associated with Leigh syndrome. Mitochondrion, 62, 13-23.

Vacchiano, V., Caporali, L., La Morgia, C., Carbonelli, M., Amore, G., Bartolomei, I., ... & Carelli, V. (2021). The m. 3890G> A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes. Mitochondrion, 60, 142-149.

Xu, Y., Zhou, J., Yuan, Q., Su, J., Li, Q., Lu, X., ... & Han, J. (2021). Quantitative detection of circulating MT-ND1 as a potential biomarker for colorectal cancer. Bosnian journal of basic medical sciences, 21(5), 577.

Zhang, J., Ji, Y., Chen, J., Xu, M., Wang, G., Ci, X., ... & Guan, M. X. (2021). Assocation between Leber's hereditary optic neuropathy and MT-ND1 3460G> a mutation-induced alterations in mitochondrial function, apoptosis, and mitophagy. Investigative Ophthalmology & Visual Science, 62(9), 38-38.

Gutiérrez Cortés, N., Pertuiset, C., Dumon, E., Börlin, M., Da Costa, B., Le Guédard, M., ... & Rocher, C. (2020). Mutation m. 3395A> G in MT-ND1 leads to variable pathologic manifestations. Human Molecular Genetics, 29(6), 980-989.

Peron, C., Mauceri, R., Cabassi, T., Segnali, A., Maresca, A., Iannielli, A., ... & Tiranti, V. (2020). Generation of a human iPSC line, FINCBi001-A, carrying a homoplasmic m. G3460A mutation in MT-ND1 associated with Leber’s Hereditary optic Neuropathy (LHON). Stem Cell Research, 48, 101939.

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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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