NDUFAF3 Antibody (PACO59437)
- SKU:
- PACO59437
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | NDUFAF3 Antibody (PACO59437) |
| Antibody SKU: | PACO59437 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human NADH dehydrogenase [ubiquinone] 1 α subcomplex assembly factor 3 protein (91-184AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | IHC image of PACO59437 diluted at 1:100 and staining in paraffin-embedded human appendix tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
 | IHC image of PACO59437 diluted at 1:100 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
| Background: | Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). |
| Synonyms: | NADH dehydrogenase [ubiquinone] 1 α subcomplex assembly factor 3, NDUFAF3, C3orf60 |
| UniProt Protein Function: | NDUFAF3: Essential factor for the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). Defects in NDUFAF3 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. 2 isoforms of the human protein are produced by alternative splicing.Protein type: MitochondrialChromosomal Location of Human Ortholog: 3p21.31Cellular Component: mitochondrial inner membrane; nucleusMolecular Function: protein bindingBiological Process: mitochondrial respiratory chain complex I assemblyDisease: Mitochondrial Complex I Deficiency |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a mitochondrial complex I assembly protein that interacts with complex I subunits. Mutations in this gene cause mitochondrial complex I deficiency, a fatal neonatal disorder of the oxidative phosphorylation system. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2009] |
| UniProt Code: | Q9BU61 |
| NCBI GenInfo Identifier: | 74733183 |
| NCBI Gene ID: | 25915 |
| NCBI Accession: | Q9BU61.1 |
| UniProt Secondary Accession: | Q9BU61 |
| UniProt Related Accession: | Q9BU61 |
| Molecular Weight: | 13,842 Da |
| NCBI Full Name: | NADH dehydrogenase |
| NCBI Synonym Full Names: | NADH:ubiquinone oxidoreductase complex assembly factor 3 |
| NCBI Official Symbol: | NDUFAF3 |
| NCBI Official Synonym Symbols: | 2P1; E3-3; C3orf60 |
| NCBI Protein Information: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 |
| UniProt Protein Name: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 |
| UniProt Synonym Protein Names: | |
| Protein Family: | NADH dehydrogenase |
| UniProt Gene Name: | NDUFAF3 |
| UniProt Entry Name: | NDUF3_HUMAN |
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