|别 名||Cystatin 3; Cystatin-3; CST 3;CST3; CST-3; Cystatin C; AD 8; AD8; Amyloid angiopathy and cerebral hemorrhage; Cst 3; Cst3; CST3 protein; Cystatin 3; Cystatin3; CystatinC; Gamma trace; HCCAA; Neuroendocrine basic polypeptide; Post gamma globulin; ARMD11; MGC117328; CYSC.|
|规格价格||50ul/860元 购买 100ul/1580元 购买 大包装/询价|
|说 明 书||50ul 100ul|
|研究领域||肿瘤 心血管 细胞生物 免疫学 发育生物学|
|克 隆 号||9H8|
|产品应用||WB=1:500-1000 ELISA=1:500-1000 IHC-P=1:500-1000 IHC-F=1:500-1000 ICC=1:100-500 IF=1:500-1000 （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||14kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||Recombinant human Cystatin C Protein:|
|纯化方法||affinity purified by Protein G|
|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.|
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins(stefins), type 2 cystatins and the kininogens. The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions, where they appear to provide protective functions. The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in the cystatin locus and encodes the most abundant extracellular inhibitor of cysteine proteases, which is found in high concentrations in biological fluids and is expressed in virtually all organs of the body. A mutation in this gene has been associated with amyloid angiopathy. Expression of this protein in vascular wall smooth muscle cells is severely reduced in both atherosclerotic and aneurysmal aortic lesions, establishing its role in vascular disease. [provided by RefSeq].
As an inhibitor of cysteine proteinases, this protein is thought to serve an important physiological role as a local regulator of this enzyme activity.
Expressed in submandibular and sublingual saliva but not in parotid saliva (at protein level). Expressed in various body fluids, such as the cerebrospinal fluid and plasma. Expressed in highest levels in the epididymis, vas deferens, brain, thymus, and ovary and the lowest in the submandibular gland.
Defects in CST3 are the cause of amyloidosis type 6 (AMYL6) [MIM:105150]; also known as hereditary cerebral hemorrhage with amyloidosis (HCHWA), cerebral amyloid angiopathy (CAA) or cerebroarterial amyloidosis Icelandic type. AMYL6 is a hereditary generalized amyloidosis due to cystatin C amyloid deposition. Cystatin C amyloid accumulates in the walls of arteries, arterioles, and sometimes capillaries and veins of the brain, and in various organs including lymphoid tissue, spleen, salivary glands, and seminal vesicles. Amyloid deposition in the cerebral vessels results in cerebral amyloid angiopathy, cerebral hemorrhage and premature stroke. Cystatin C levels in the cerebrospinal fluid are abnormally low.
Belongs to the cystatin family.
Entrez Gene: 1471 Human
Entrez Gene: 13010 Mouse
Entrez Gene: 25307 Rat
Omim: 604312 Human
SwissProt: P01034 Human
SwissProt: P21460 Mouse
SwissProt: P14841 Rat
Unigene: 304682 Human
Unigene: 4263 Mouse
Unigene: 106351 Rat
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
胱抑素C（ cystatin c）是一种半胱氨酸蛋白酶抑制剂,也被称为γ-微量蛋白及γ-后球蛋白，广泛存在于各种组织的有核细胞和体液中,是一种低分子量、碱性非糖化蛋白质，分子量为13.3KD,由122个氨基酸残基组成,可由机体所有有核细胞产生，产生率恒定。循环中的胱抑素c仅经肾小球滤过而被清除，是一种反映肾小球滤过率变化的内源性标志物,并在近曲小管重吸收，但重吸收后被完全代谢分解，不返回血液，因此，其血中浓度由肾小球滤过决定，而不依赖任何外来因素，如性别、年龄、饮食的影响，是一种反映肾小球滤过率变化的理想同源性标志物。