- Catalog number
- PA2232-Dylight550
- Size / count
- 100 µg
- Dry ice
- No
- Hazardous
- No
- Discontinued
- Yes
- Background
- Apoptosis-inducing factor 1, mitochondrial, also known as AIF or PDCD8 is a protein that in humans is encoded by the AIFM1 gene. AIFM1 gene is mapped to Xq26.1 based on an alignment of the AIFM1 sequence with the genomic sequence. This gene encodes a flavoprotein essential for nuclear disassembly in apoptotic cells, and it is found in the mitochondrial intermembrane space in healthy cells. Induction of apoptosis results in the translocation of this protein to the nucleus where it affects chromosome condensation and fragmentation. In addition, this gene product induces mitochondria to release the apoptogenic proteins cytochrome c and caspase-9. Mutations in this gene cause combined oxidative phosphorylation deficiency 6, which results in a severe mitochondrial encephalomyopathy. A related pseudogene has been identified on chromosome 10.
- Synonyms
- Apoptosis-inducing factor 1, mitochondrial;1.1.1.-;Programmed cell death protein 8;AIFM1;AIF, PDCD8;
- Gene Name
- Apoptosis-inducing factor 1, mitochondrial
- UniProt
- O95831
- Host
- Rabbit
- Reactivity
- Rat,Mouse,Human
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human AIF, identical to the related rat and mouse sequences.
- Clonality
- Polyclonal
- Tissue Specificity
- Detected in muscle and skin fibroblasts (at protein level). Isoform 5 is frequently down-regulated in human cancers. .
- Applications
- IHC,ICC,WB
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Functions both as NADH oxidoreductase and as regulator of apoptosis. In response to apoptotic stimuli, it is released from the mitochondrion intermembrane space into the cytosol and to the nucleus, where it functions as a proapoptotic factor in a caspase-independent pathway. In contrast, functions as an antiapoptotic factor in normal mitochondria via its NADH oxidoreductase activity. The soluble form (AIFsol) found in the nucleus induces 'parthanatos' i.e. caspase-independent fragmentation of chromosomal DNA. Interacts with EIF3G,and thereby inhibits the EIF3 machinery and protein synthesis, and activates casapse-7 to amplify apoptosis. Plays a critical role in caspase- independent, pyknotic cell death in hydrogen peroxide-exposed cells. Binds to DNA in a sequence-independent manner. .
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 1. Ghezzi, D., Sevrioukova, I., Invernizzi, F., Lamperti, C., Mora, M., D'Adamo, P., Novara, F., Zuffardi, O., Uziel, G., Zeviani, M. Severe X-linked mitochondrial encephalomyopathy associated with a mutation in apoptosis-inducing factor. Am. J. Hum. Genet. 86: 639-649, 2010.
2. Rinaldi, C., Grunseich, C., Sevrioukova, I. F., Schindler, A., Horkayne-Szakaly, I., Lamperti, C., Landoure, G., Kennerson, M. L., Burnett, B. G., Bonnemann, C., Biesecker, L. G., Ghezzi, D., Zeviani, M., Fischbeck, K. H. Cowchock syndrome is associated with a mutation in apoptosis-inducing factor. Am. J. Hum. Genet. 91: 1095-1102, 2012.
3. Yu, S.-W., Andrabi, S. A., Wang, H., Kim, N. S., Poirier, G. G., Dawson, T. M., Dawson, V. L. Apoptosis-inducing factor mediates poly(ADP-ribose) (PAR) polymer-induced cell death. Proc. Nat. Acad. Sci. 103: 18314-18319, 2006.
- Storage Conditions
- Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 66901 MW
- Observed Molecular Weight
- 60 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Immunocytochemistry , 0.5-1μg/ml, Human, Mouse, Rat<br>Immunohistochemistry (Frozen Section), 0.5-1μg/ml, Rat, Human, Mouse<br>Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, Rat, Mouse, By Heat<br>Western blot, 0.1-0.5μg/ml, Human, Mouse, Rat<br>
- Subcellular Location
- Mitochondrion intermembrane space. Mitochondrion inner membrane. Cytoplasm. Nucleus. Cytoplasm, perinuclear region. Proteolytic cleavage during or just after translocation into the mitochondrial intermembrane space (IMS) results in the formation of an inner-membrane-anchored mature form (AIFmit). During apoptosis, further proteolytic processing leads to a mature form, which is confined to the mitochondrial IMS in a soluble form (AIFsol). AIFsol is released to the cytoplasm in response to specific death signals, and translocated to the nucleus, where it induces nuclear apoptosis. Colocalizes with EIF3G in the nucleus and perinuclear region.
- Sequence Similarities
- Belongs to the FAD-dependent oxidoreductase family.
- Protein Name
- Apoptosis-inducing factor 1, mitochondrial
