NDUFAF6 Polyclonal Antibody
Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application ![]()
| IHC-P, IHC-F, IF, ICC, E |
---|---|
Primary Accession | Q330K2 |
Reactivity | Rat |
Host | Rabbit |
Clonality | Polyclonal |
Calculated MW | 33 KDa |
Physical State | Liquid |
Immunogen | KLH conjugated synthetic peptide derived from human NDUFAF6 |
Epitope Specificity | 201-300/333 |
Isotype | IgG |
Purity | affinity purified by Protein A |
Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
SUBCELLULAR LOCATION | Isoform 1: Mitochondrion inner membrane. Note: Peripherally localized on the matrix face of the mitochondrial inner membrane. Ref.7 Isoform 2: Cytoplasm. Nucleus |
SIMILARITY | Belongs to the NDUFAF6 family. |
DISEASE | Mitochondrial complex I deficiency (MT-C1D) [MIM:252010]: A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations 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. Note: The disease is caused by mutations affecting the gene represented in this entry. |
Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
Background Descriptions | This gene encodes a protein that localizes to mitochondria and contains a predicted phytoene synthase domain. The encoded protein plays an important role in the assembly of complex I (NADH-ubiquinone oxidoreductase) of the mitochondrial respiratory chain through regulation of subunit ND1 biogenesis. Mutations in this gene are associated with complex I enzymatic deficiency. [provided by RefSeq, Nov 2011] |
Gene ID | 137682 |
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Other Names | NADH dehydrogenase (ubiquinone) complex I, assembly factor 6, Putative phytoene synthase, NDUFAF6, C8orf38 |
Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
Format | 0.01M TBS(pH7.4), 0.09% (W/V) sodium azide and 50% Glyce |
Storage | Store at -20 ℃ 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 ℃. |
Name | NDUFAF6 |
---|---|
Synonyms | C8orf38 |
Function | Involved in the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I) at early stages. May play a role in the biogenesis of complex I subunit MT-ND1. |
Cellular Location | [Isoform 1]: Mitochondrion inner membrane. Note=Peripherally localized on the matrix face of the mitochondrial inner membrane |
Tissue Location | Widely expressed. A lower expression is observed in lung and kidney compared to heart, muscle and liver (PubMed:27466185) In the kidney, expression is high in the basal zone of the proximal tubular cells (PubMed:27466185). |

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