肌萎缩侧索硬化症相关蛋白FIG4抗体

英文名称    FIG4    

中文名称    肌萎缩侧索硬化症相关蛋白FIG4抗体    

别    名    5-bisphosphate 5-phosphatase; Fig4; FIG4_HUMAN; KIAA0274; ALS11; Phosphatidylinositol 3; hSac3; Phosphatidylinositol 3,5 bisphosphate 5 phosphatase; Polyphosphoinositide phosphatase; SAC domain containing protein 3; SAC domain-containing protein 3; SAC3.    

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物  神经生物学  信号转导      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Pig, Sheep, Monkey,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 肌萎缩侧索硬化症相关蛋白FIG4抗体（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.    

分 子 量    104kDa    

细胞定位    细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human FIG4 (1-100aa)    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    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.    

肌萎缩侧索硬化症相关蛋白FIG4抗体产品介绍    background:

The protein encoded by this gene belongs to the SAC domain-containing protein gene family. The SAC domain, approximately 400 amino acids in length and consisting of seven conserved motifs, has been shown to possess phosphoinositide phosphatase activity. The yeast homolog, Sac1p, is involved in the regulation of various phosphoinositides, and affects diverse cellular functions such as actin cytoskeleton organization, Golgi function, and maintenance of vacuole morphology. Membrane-bound phosphoinositides function as signaling molecules and play a key role in vesicle trafficking in eukaryotic cells. Mutations in this gene have been associated with Charcot-Marie-Tooth disease, type 4J. [provided by RefSeq, Jul 2008]

Function:
The PI(3,5)P2 regulatory complex regulates both the synthesis and turnover of phosphatidylinositol-3,5-bisphosphate (PtdIns(3,5)P2). In vitro, hydrolyzes all three D5-phosphorylated polyphosphoinositide substrates in the order PtdIns(4,5)P2 > PtdIns(3,5)P2 > PtdIns(3,4,5)P3. Plays a role in the biogenesis of endosome carrier vesicles (ECV) / multivesicular bodies (MVB) transport intermediates from early endosomes.

Subcellular Location:
Endosome membrane. Localization requires VAC14 and PIKFYVE.

DISEASE:
Defects in FIG4 are the cause of Charcot-Marie-Tooth disease type 4J (CMT4J) [MIM:611228]. CMT4J is a recessive demyelinating, severe form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.
Defects in FIG4 are the cause of amyotrophic lateral sclerosis type 11 (ALS11) [MIM:612577]. ALS is a neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10%.

Similarity:
Contains 1 SAC domain.

Database links:

Entrez Gene: 9896 Human

Entrez Gene: 103199 Mouse

Entrez Gene: 309855 Rat

Omim: 609390 Human

SwissProt: Q92562 Human

SwissProt: Q91WF7 Mouse

Unigene: 529959 Human

Unigene: 277242 Mouse

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

肌萎缩侧索硬化症相关蛋白FIG4抗体

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hz-5441R phospho-IKK beta(S474)  磷酸化KB抑制蛋白激酶β抗体

hz-5442R phospho-IKK beta(Ser476)  磷酸化KB抑制蛋白激酶β抗体

hz-3227R phospho-IKK gamma (Ser85)  磷酸化KB抑制蛋白激酶γ抗体

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hz-0245R IKI3 family protein  拟南芥IKI3抗体

hz-2428R IL-9  白介素9抗体

hz-0698R IL-10  白细胞介素-10抗体

hz-1827R IL-11  白介素11抗体

hz-2459R IL-10RA  白细胞介素-10受体a抗体

hz-2602R IL-10RB/CRFB4  白细胞介素-10受体β抗体

hz-0767R IL-12  白介素12抗体

hz-1789R IL-12  白介素12抗体

hz-2603R IL-12RB1/CD212  白细胞介素-12受体β1抗体

hz-2604R IL12RB2  白细胞介素-12受体β2抗体

hz-0560R IL-13  白介素13抗体

hz-1771R IL-14/Taxilin alpha  白介素14抗体

hz-2605R IL-15RA  白介素15受体α抗体

hz-1829R IL-15  白介素15抗体

hz-1830R IL-16  白介素16抗体

hz-2460R IL-13Ra1  白细胞介素-13受体a1抗体

hz-2461R IL-13Ra2  白细胞介素-13受体a2抗体

hz-0768R IL-16/LCF  白介素-16抗体

hz-1183R IL-17A  白介素-17抗体

hz-2140R IL-17  白介素-17抗体

hz-2606R IL-17R/IL-17RA/CD217  白介素17受体抗体

hz-2607R IL-17RC/IL-17RL  白介素17受体C抗体