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Mimitin Rabbit pAb
- 产品详情
- 实验流程
Application 
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q8N183 |
| Other Accession | Q8N183 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 19856 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human Mimitin |
| Epitope Specificity | 71-169/169 |
| 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 NDUFA12 subunit family. |
| SUBUNIT | Mitochondrion. |
| DISEASE | Defects in NDUFAF2 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 | NADH:ubiquinone oxidoreductase (complex I) catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q) in the first step of the mitochondrial respiratory chain, resulting in the translocation of protons across the inner mitochondrial membrane. This gene encodes a complex I assembly factor. Mutations in this gene cause progressive encephalopathy resulting from mitochondrial complex I deficiency. |
| Gene ID | 91942 |
|---|---|
| Other Names | B17.2L; MC1DN10; MMTN; NDUFA12L; mimitin; 1810058I14Rik; RGD1560158; NDUF2_HUMAN; NDUFAF2; B17.2-like (B17.2L); Myc-induced mitochondrial protein (MMTN); NDUFA12-like protein; NDUF2_MOUSE; NADH:ubiquinone oxidoreductase complex assembly factor 2; NDUFA12-like; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 2; NADH dehydrogenase (ubiquinone) complex I, assembly factor 2; Myc-induced mitochondrial protein |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| 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 | NDUFAF2 |
|---|---|
| Synonyms | NDUFA12L |
| Function | Acts as a molecular chaperone for mitochondrial complex I assembly (PubMed:16200211, PubMed:19384974). 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 (PubMed:16200211, PubMed:27626371). Is involved in the initial steps of cilia formation, including removal of CP110 from the mother centrioles, docking of membrane vesicles to the mother centrioles, and establishment of the transition zone (PubMed:38949024). |
| Cellular Location | Mitochondrion. |
| Tissue Location | Highly expressed in ESCC cells. Also expressed in heart, skeletal muscle, liver, and in fibroblasts |
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# AP98791
