Testing Data
(Gel: 8%SDS-PAGE Lysates (from left to right): Mouse heart and brain tissue Amount of lysate: 40ug per lane Primary antibody: 1/500 dilution Secondary antibody dilution: 1/8000 Exposure time: 30 seconds)
Rabbit anti-Human, Mouse NDUFV1 Polyclonal Antibody | anti-NDUFV1 antibody
NDUFV1 Antibody
Testing Data
(Gel: 8%SDS-PAGE Lysates (from left to right): Mouse heart and brain tissue Amount of lysate: 40ug per lane Primary antibody: 1/500 dilution Secondary antibody dilution: 1/8000 Exposure time: 30 seconds)
Testing Data
(Gel: 8%SDS-PAGE Lysates (from left to right): Mouse heart and brain tissue Amount of lysate: 40ug per lane Primary antibody: 1/500 dilution Secondary antibody dilution: 1/8000 Exposure time: 30 seconds)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
NDUFV1: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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. Defects in NDUFV1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, 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. Belongs to the complex I 51 kDa subunit family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Oxidoreductase; Mitochondrial; EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation; EC 1.6.99.3
Chromosomal Location of Human Ortholog: 11q13
Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I
Molecular Function: NADH dehydrogenase (ubiquinone) activity; FMN binding; metal ion binding; 4 iron, 4 sulfur cluster binding; NAD binding
Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone
Disease: Mitochondrial Complex I Deficiency
Research Articles on NDUFV1
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Product Notes
The NDUFV1 ndufv1 (Catalog #AAA9410786) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The NDUFV1 Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's NDUFV1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western blotting: 1:500-1:2000. Researchers should empirically determine the suitability of the NDUFV1 ndufv1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "NDUFV1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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