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NDUFA1 Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | O15239 |
| Other Accession | O15239 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 8072 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human NDUFA1 |
| Epitope Specificity | 31-70/70 |
| 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 NDUFA1 subunit family. |
| SUBUNIT | Complex I is composed of 45 different subunits. |
| DISEASE | Defects in NDUFA1 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | NDUFA1 (NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1) is an essential component of the multisubunit NADH ubiquinone oxidoreductase (complex 1), the first enzyme complex in the mitochondrial respiratory chain. Complex I transfers electrons from NADH to the respiratory chain via ubiquinone. |
| Gene ID | 4694 |
|---|---|
| Other Names | NDUA1_HUMAN; NDUFA1; Complex I-MWFE (CI-MWFE); NADH-ubiquinone oxidoreductase MWFE subunit; CI-MWFE; MC1DN12; MWFE; ZNF183; NADH:ubiquinone oxidoreductase subunit A1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1 (7.5kD, MWFE); NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa; NADH:ubiquinone oxidoreductase (complex 1); type I dehydrogenase; complex I MWFE subunit |
| Dilution | 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 | NDUFA1 |
|---|---|
| Function | Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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. |
| Cellular Location | Mitochondrion inner membrane; Single-pass membrane protein; Matrix side |
| Tissue Location | Primarily expressed in heart and skeletal muscle. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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Cat# AP100146
