中/英文名称：

调亡诱导因子抗体   / Anti- AIF

别名：

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.

产品编号：

zy10039

规格：

0.1ml    0.2ml

抗体来源：

Mouse

浓    度：

1mg/ml

缓冲液：

0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1%   Sodium azide；Reconstitute with 0.1ml sterile distilled water.

克隆类型：

Polyclonal

亚 型：

IgG

免 疫 原：

KLH conjugated synthetic peptide derived from   human AIF

交叉反应：

Human, Mouse, Rat, Chicken, Dog, Pig, Cow,   Rabbit, Sheep

状态：

Lyophilized or Liquid

分 子 量：

Predicted molecular weight 56kDa

纯化方法：

affinity purified by Protein A

运输与储存

运输:

The product is shipped at ambient temperature.   Upon receipt, store it immediately at the temperature recommended below.

储存:

Use a manual defrost freezer and avoid repeated   freezethaw cycles.

1 month from date of receipt, 2 to 8℃, reconstituted.

6 months from date of receipt, -20 to -70℃, reconstituted.

12 months from date of receipt, -20 to -70℃as supplied.

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.

产品应用

应用:

ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

背景:

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].

其他:

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:

Defects in AIFM1 are the cause of combined   oxidative phosphorylation deficiency type 6 (COXPD6) [MIM:300816]. It is a   mitochondrial disease resulting in a neurodegenerative disorder characterized   by psychomotor delay, hypotonia, areflexia, muscle weakness and wasting.

Similarity:

Belongs to the FAD-dependent oxidoreductase   family.

Gene   ID:9131

AIF是一种易位到细胞核诱导凋亡的线粒体蛋白, AIF可引起DNA破碎、染色质凝聚，还可诱导细胞色素C和Caspase-9从线粒体中释放出来，AIF从线粒体中的释放可被过度表达的Bcl-2（一种参与线粒体渗透的蛋白质）所抑制。