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Rabbit Anti-Cystatin-C/BF647 Conjugated antibody (bs-10876R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-10876R-BF647
英文名稱1 Rabbit Anti-Cystatin-C/BF647 Conjugated antibody
中文名稱 BF647標記的胱抑素C/半胱氨酸蛋白酶抑制劑C抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  心血管  細胞生物  免疫學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, 
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 13kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 Recombinant human Cystatin-C
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
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:
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].

Function:
As an inhibitor of cysteine proteinases, this protein is thought to serve an important physiological role as a local regulator of this enzyme activity.

Subunit:
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.

Subcellular Location:
Secreted.

DISEASE:
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.

Similarity:
Belongs to the cystatin family.

Database links:

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



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