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Rabbit Anti-AIF/BF647 Conjugated antibody (bs-0037R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0037R-BF647
英文名稱 Rabbit Anti-AIF/BF647 Conjugated antibody
中文名稱 BF647標記的調亡誘導因子抗體
別    名 Apoptosis inducing factor; Harlequin; Hq; mAIF; MGC111425; MGC5706; PDCD 8; PDCD8; Programmed cell death 8; Programmed cell death 8 isoform 1; Programmed cell death 8 isoform 2; Programmed cell death 8 isoform 3; Programmed cell death protein 8 mitochondrial; Programmed cell death protein 8 mitochondrial precursor; Striatal apoptosis inducing factor; AIFM1_HUMAN; Apoptosis-inducing factor 1, mitochondrial.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  染色質和核信號  神經生物學  細胞凋亡  細胞周期蛋白  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Pig,  (predicted: Rat, Chicken, Dog, Cow, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 57kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human AIF N-terminus
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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 °C.
產品介紹 background:
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. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 10. [provided by RefSeq, May 2010].

Function:
Probable oxidoreductase that has a dual role in controlling cellular life and death; during apoptosis, it is translocated from the mitochondria to the nucleus to function as a proapoptotic factor in a caspase-independent pathway, while in normal mitochondria, it functions as an antiapoptotic factor via its 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.

Subunit:
Interacts with XIAP/BIRC4. Interacts (via N-terminus) with EIF3G (via C-terminus).

Subcellular Location:
Mitochondrion intermembrane space. Mitochondrion inner membrane. Cytoplasm. Nucleus. Cytoplasm, perinuclear region. Note=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.

Tissue Specificity:
Isoform 5 is frequently down-regulated in human cancers.

Post-translational modifications:
Under normal conditions, a 54-residue N-terminal segment is first proteolytically removed during or just after translocation into the mitochondrial intermembrane space (IMS) by the mitochondrial processing peptidase (MPP) to form the inner-membrane-anchored mature form (AIFmit). During apoptosis, it is further proteolytically processed at amino-acid position 101 leading to the generation of the 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 in a caspase-independent manner.
Ubiquitination by XIAP/BIRC4 does not lead to proteasomal degradation. Ubiquitination at Lys-255 by XIAP/BIRC4 blocks its ability to bind DNA and induce chromatin degradation, thereby inhibiting its ability to induce cell death.

DISEASE:
Combined oxidative phosphorylation deficiency 6 (COXPD6) [MIM:300816]: A mitochondrial disease resulting in a neurodegenerative disorder characterized by psychomotor delay, hypotonia, areflexia, muscle weakness and wasting. Some patients manifest prenatal ventriculomegaly and severe postnatal encephalomyopathy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the FAD-dependent oxidoreductase family.

Database links:

Entrez Gene: 51060 Human

Entrez Gene: 9131 Human

Entrez Gene: 26926 Mouse

Entrez Gene: 83533 Rat

Omim: 300169 Human

SwissProt: O95831 Human

SwissProt: Q9Z0X1 Mouse

SwissProt: Q9JM53 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

AIF是一種易位到細胞核誘導凋亡的線粒體蛋白, AIF可引起DNA破碎、染色質凝聚,還可誘導細胞色素C和Caspase-9從線粒體中釋放出來,AIF從線粒體中的釋放可被過度表達的Bcl-2(一種參與線粒體滲透的蛋白質)所抑制。
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