Mouse Anti-NDUFV1 Recombinant Antibody (4A7) (CBMAB-N1700-WJ)

Name: Mouse Anti-NDUFV1 Recombinant Antibody (4A7)
SKU: CBMAB-N1700-WJ
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

4A7

Application

ELISA, WB

Specificity

Human

Antibody Isotype

IgG2b, 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:Ubiquinone Oxidoreductase Core Subunit V1

Introduction

The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]

Entrez Gene ID

4723

UniProt ID

P49821

Alternative Names

NADH:Ubiquinone Oxidoreductase Core Subunit V1; NADH Dehydrogenase [Ubiquinone] Flavoprotein 1, Mitochondrial; NADH Dehydrogenase (Ubiquinone) Flavoprotein 1, 51kDa; NADH-Ubiquinone Oxidoreductase 51 KDa Subunit; Complex I 51kDa Subunit; UQOR1; Mitochondrial NADH:Ubiquinone Oxidoreductase 51 Kda Subunit; Mitochondrial NADH Dehydrogenase Ubiquinone Flavoprotein 1; NADH Dehydrogenase (Ubiquinone) Flavoprotein 1 (51kD); Complex I, Mitochondrial Respiratory Chain;

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.

Biological Process

Aerobic respiration Source: ComplexPortal
Mitochondrial ATP synthesis coupled electron transport Source: CAFA
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 4 (MC1DN4):
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. MC1DN4 transmission pattern is consistent with autosomal recessive inheritance.

Ma, X., Han, Y., Liu, K., Bai, Y., Gao, H., Hou, Y., & Bai, G. (2023). Chemical proteomics combined with metabonomics reveals berberine targets NDUFV1 of complex I in the respiratory chain to regulate energy metabolism. Chinese Chemical Letters, 34(3), 107595.

Li, L., Zhang, L., Cao, Y., Chen, X., Gong, H., Ma, Y., ... & Huang, X. (2023). NDUFV1 attenuates renal ischemia–reperfusion injury by improving mitochondrial homeostasis. Journal of Cellular and Molecular Medicine.

Tang, X., Xu, W., Song, X., Ye, H., Ren, X., Yang, Y., ... & Lan, X. (2022). Compound heterozygous mutations of NDUFV1 identified in a child with mitochondrial complex I deficiency. Genes & Genomics, 44(6), 691-698.

Wang, R., Kairen, C., Li, L., Zhang, L., Gong, H., & Huang, X. (2022). Overexpression of NDUFV1 alleviates renal damage by improving mitochondrial function in unilateral ureteral obstruction model mice. Cell Biology International, 46(3), 381-390.

Xue, W., Li, X., Li, W., Wang, Y., Jiang, C., Zhou, L., ... & Xu, Q. (2022). Intracellular CYTL1, a novel tumor suppressor, stabilizes NDUFV1 to inhibit metabolic reprogramming in breast cancer. Signal Transduction and Targeted Therapy, 7(1), 35.

Zanette, V., Valle, D. D., Telles, B. A., Robinson, A. J., Monteiro, V., Santos, M. L. S., ... & Benincá, C. (2021). NDUFV1 mutations in complex I deficiency: Case reports and review of symptoms. Genetics and Molecular Biology, 44, e20210149.

Borna, N. N., & Okazaki, Y. (2021). A typical phenotypes due to NDUFV1 mutations. Pediatric Urology Case Reports, 8(5), 107-113.

Li, B., Yang, Y., Wang, Y., Zhang, J., Ding, J., Liu, X., ... & Sun, C. (2021). Acetylation of NDUFV1 induced by a newly synthesized HDAC6 inhibitor HGC rescues dopaminergic neuron loss in Parkinson models. Iscience, 24(4).

Finsterer, J., & Zarrouk-Mahjoub, S. (2019). Cystic Leucoencephalopathy in NDUFV1 Mutation: Correspondence. The Indian Journal of Pediatrics, 86, 206-207.

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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

Isotype control

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

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