Western Blot (WB)
(Human nuclear lysates)
Rabbit NDUFS1 Polyclonal Antibody | anti-NDUFS1 antibody
NDUFS1 antibody - middle region
Western Blot (WB)
(Human nuclear lysates)
Western Blot (WB)
(Human nuclear lysates)
Western Blot (WB)
(Lanes:Lane1: 30 ug Human liverLane2: 30 ug Rat liverLane3: 30 ug Mouse (wild-type) liverLane4: 30 ug Mouse [AMPK alpha 1/2(-/-)] liverLane5: 30 ug Human muscleLane6: 30 ug Rat muscleLane7: 30 ug Mouse musclePrimary Antibody Dilution:1:1000Secondary Antibody:Secondary Antibody Dilution:1:10000Gene Name:NDUFS1Submitted by:Dr. Bruno Guigas, PhD, Dept. of Molecular Cell Biology, Leiden University Medical Center)
Western Blot (WB)
(Lanes:Lane1: 30 ug Human liverLane2: 30 ug Rat liverLane3: 30 ug Mouse (wild-type) liverLane4: 30 ug Mouse [AMPK alpha 1/2(-/-)] liverLane5: 30 ug Human muscleLane6: 30 ug Rat muscleLane7: 30 ug Mouse musclePrimary Antibody Dilution:1:1000Secondary Antibody:Secondary Antibody Dilution:1:10000Gene Name:NDUFS1Submitted by:Dr. Bruno Guigas, PhD, Dept. of Molecular Cell Biology, Leiden University Medical Center)
Western Blot (WB)
(WB Suggested Anti-NDUFS1 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: Fetal Liver)
Western Blot (WB)
(WB Suggested Anti-NDUFS1 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: Fetal Liver)
Target Description: The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2011]
Uniprot Description
NDUFS1: 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. This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Defects in NDUFS1 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 75 kDa subunit family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Oxidoreductase; Energy Metabolism - oxidative phosphorylation; EC 1.6.5.3; Mitochondrial; EC 1.6.99.3
Chromosomal Location of Human Ortholog: 2q33-q34
Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial intermembrane space; mitochondrial respiratory chain complex I
Molecular Function: 2 iron, 2 sulfur cluster binding; protein binding; electron carrier activity; NADH dehydrogenase (ubiquinone) activity; 4 iron, 4 sulfur cluster binding; metal ion binding
Biological Process: ATP metabolic process; cellular metabolic process; apoptotic mitochondrial changes; mitochondrial electron transport, NADH to ubiquinone; regulation of mitochondrial membrane potential; cellular respiration
Disease: Mitochondrial Complex I Deficiency
Research Articles on NDUFS1
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Product Notes
The NDUFS1 ndufs1 (Catalog #AAA3212877) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The NDUFS1 antibody - middle region reacts with Cow, Dog, Goat, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's NDUFS1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the NDUFS1 ndufs1 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. The amino acid sequence is listed below: Synthetic peptide located within the following region: TPPGLAREDW KIIRALSEIA GMTLPYDTLD QVRNRLEEVS PNLVRYDDIE. It is sometimes possible for the material contained within the vial of "NDUFS1, 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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