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NDUFV1 Rabbit pAb
- 产品详情
- 实验流程
Application 
| WB, IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P49821 |
| Other Accession | P49821 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 50817 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human NDUFV1 |
| Epitope Specificity | 41-140/464 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the complex I 51 kDa subunit family. |
| SUBUNIT | Complex I is composed of 45 different subunits. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme. |
| DISEASE | Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 4723 |
|---|---|
| Other Names | CI-51K; CI51KD; MC1DN4; UQOR1; CI-51kD; NDUV1_BOVIN; NDUFV1; Complex I-51kD (CI-51kD); NADH dehydrogenase flavoprotein 1; NADH-ubiquinone oxidoreductase 51 kDa subunit; 7.1.1.2; NDUV1_HUMAN; NDUV1_MOUSE; Q5XIH3_RAT; NADH:ubiquinone oxidoreductase core subunit V1; NADH dehydrogenase (ubiquinone) flavoprotein 1 (51kD); NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa; complex I 51kDa subunit; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | NDUFV1 (HGNC:7716) |
|---|---|
| Synonyms | UQOR1 |
| 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:28844695). Part of the peripheral arm of the enzyme, where the electrons from NADH are accepted by flavin mononucleotide (FMN) and then passed along a chain of iron-sulfur clusters by electron tunnelling to the final acceptor ubiquinone (PubMed:28844695). Contains FMN, which is the initial electron acceptor as well as one iron-sulfur cluster (PubMed:28844695). |
| Cellular Location | Mitochondrion inner membrane {ECO:0000250|UniProtKB:P25708}; Peripheral membrane protein {ECO:0000250|UniProtKB:P25708}; Matrix side {ECO:0000250|UniProtKB:P25708} |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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¥ 1,250.00
Cat# AP98503
