Anti-CCCM2抗体Cerebral cavernous malformation （CCM） is an autosomal dominant or sporadic neurovascular disease marked by vascular anomalies located mostly in the central

产品编号 yb-11817R
英文名称 Anti-CCCM2抗体
中文名称 脑海绵状血管畸形蛋白2抗体
别    名 Malcavernin; C7orf22; Ccm2; CCM2_HUMAN; Cerebral cavernous malformation 2; Cerebral cavernous malformations 2 protein; Malcavernin; MGC4067; MGC4607; MGC74868; PP10187.
 Anti-CCCM2抗体   
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Cow, Horse, Sheep, h,
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 （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 49kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Malcavernin
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
 
PubMed PubMed
产品介绍 background:
Cerebral cavernous malformation (CCM) is an autosomal dominant or sporadic neurovascular disease marked by vascular anomalies located mostly in the central nervous system that can cause stroke, seizures, cerebral hemorrhages, headaches and focal neurologic deficits. CCM is caused by mutations in one of three genes: CCM1, CCM2 or CCM3. CCM1 encodes the protein KRIT1, CCM2 encodes the protein Malcavernin and CCM3 shares its name with the protein it encodes. Malcavernin, also designated cerebral cavernous malformations 2 protein, is a scffolding protein for MEK kinase-3. Like KRIT1, Malcavernin is expresed in a variety of human organs including the arterial vascular endothelium, pyramidal neurons, astrocytes and their foot processes. In addition, Malcavernin is expressed in various epithelial cells that are required for the formation of the blood-organ barrier. Malcavernin is localized to the cytoplasm but is known to shuttle to and from the nucleus. Due to its lack of a nuclear export signal or nuclear localization signal, it is believed that Malcavernin accomplishes this shuttling via an attachment to KRIT1, which contains a nuclear localization signal. Two isoforms exist for Malcavernin. Isoform 1 represents the full length protein while isoform 2 contains an alternative four amino acid sequence rather than the first 10 residues of isoform 1.

Function:
May function as a scaffold protein for MAP2K3-MAP3K3 signaling. Seems to play a major role in the modulation of MAP3K3-dependent p38 activation induced by hyperosmotic shock.

Subunit:
Part of a complex with MAP2K3, MAP3K3 and RAC1. Binds RAC1 directly and independently of its nucleotide-bound state (By similarity). Interacts with PDCD10. Interacts with HEG1 and KRIT1; KRIT1 greatly facilitates the interaction with HEG1 (By similarity).