The NDUFAF3 Polyclonal Antibody (PAC05937) is a valuable tool for researchers studying NDUFAF3, a protein involved in mitochondrial complex I assembly and function. This antibody, produced in rabbits, is highly specific for detecting NDUFAF3 in human samples and has been validated for use in Western blot applications. By targeting NDUFAF3, researchers can investigate its role in mitochondrial function and its potential implications in diseases related to mitochondrial dysfunction, such as neurodegenerative disorders and metabolic diseases.
The NDUFAF3 Polyclonal Antibody is an essential tool for studies aiming to uncover the molecular mechanisms underlying mitochondrial complex I assembly and its impact on cellular health.
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)
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).
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]