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apoptosis inducing factor mitochondria associated 1 OKDB#: 3976
 Symbols: AIFM1 Species: human
 Synonyms: AIF, AUNX1, CMT2D, CMTX4, COWCK, DFNX5, NADMR, NAMSD, PDCD8, COXPD6, SEMDHL  Locus: Xq26.1 in Homo sapiens


For retrieval of Nucleotide and Amino Acid sequences please go to: OMIM Entrez Gene
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General Comment NCBI Summary: 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 (COXPD6), a severe mitochondrial encephalomyopathy, as well as Cowchock syndrome, also known as X-linked recessive Charcot-Marie-Tooth disease-4 (CMTX-4), a disorder resulting in neuropathy, and axonal and motor-sensory defects with deafness and cognitive disability. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 10. [provided by RefSeq, Aug 2015]
General function Cell death/survival
Comment
Cellular localization Mitochondrial
Comment
Ovarian function Follicle atresia
Comment Knock-down of apoptosis inducing factor gene protects endoplasmic reticulum stress-mediated goat granulosa cell apoptosis. Yang D et al. (2017) The apoptosis of granulosa cells is the main cause of follicular atresia, and endoplasmic reticulum (ER) stress is involved in the apoptosis of granulosa cells. Apoptosis inducing factor (AIF) mediates caspase-independent apoptosis and causes chromatin condensation and DNA fragmentation, but its role in ER stress-mediated granulosa cell apoptosis during goat follicular atresia remains largely unknown. The aim of this study was to investigate the function of AIF in the apoptosis of goat granulosa cells mediated by ER stress. The results of immunohistochemical and Western blot analyses demonstrated that AIF was mainly located in granulosa cells, and the expression of AIF significantly increased during follicular atresia. Then, AIF-short hairpin RNA recombinant lentiviral vectors were constructed successfully and transfected into human telomerase reverse transcriptase-goat granulosa cells (hTERT-GGCs). Real-time quantitative polymerase chain reaction and Western blot analysis confirmed that AIF was effectively knocked down in hTERT-GGCs. Flow cytometry results showed that the knockdown of AIF in hTERT-GGCs reduced apoptosis due to serum starvation or thapsigargin (Tg) treatment. In addition, AIF depletion changed the expression of related molecular marker molecules of ER stress under Tg treatment. In conclusion, AIF may serve as a key factor during follicular atresia, and AIF depletion protects ER stress-mediated goat granulosa cell apoptosis.//////////////////
Expression regulated by
Comment
Ovarian localization Granulosa
Comment
Follicle stages
Comment
Phenotypes POF (premature ovarian failure)
Mutations 2 mutations

Species: None
Mutation name:
type: None
fertility: None
Comment:

Species: mouse
Mutation name:
type: null mutation
fertility: embryonic lethal
Comment: Loss of Aif function causes cell death in the mouse embryo, but the temporal progression of patterning is normal. Brown D et al. (2006) Apoptosis-inducing factor (AIF) is an evolutionarily conserved, ubiquitously expressed flavoprotein with NADH oxidase activity that is normally confined to mitochondria. In mammalian cells, AIF is released from mitochondria in response to apoptotic stimuli and translocates to the nucleus where it is thought to bind DNA and contribute to chromatinolysis and cell death in a caspase-independent manner. Here we describe the consequences of inactivating Aif in the early mouse embryo. Unexpectedly, we found that both the apoptosis-dependent process of cavitation in embryoid bodies and apoptosis associated with embryonic neural tube closure occur in the absence of AIF, indicating that Aif function is not required for apoptotic cell death in early mouse embryos. By embryonic day 9 (E9), loss of Aif function causes abnormal cell death, presumably because of reduced mitochondrial respiratory chain complex I activity. Because of this cell death, Aif null embryos fail to increase significantly in size after E9. Remarkably, patterning processes continue on an essentially normal schedule, such that E10 Aif null embryos with only approximately 1/10 the normal number of cells have the same somite number as their wild-type littermates. These observations show that pattern formation in the mouse can occur independent of embryo size and cell number.//////////////////

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created: Feb. 9, 2009, 9:14 p.m. by: jeehyeon   email:
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last update: May 21, 2020, 10:07 a.m. by: hsueh    email:



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