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Product Name Cystatin-C(8F14) Chinese Name 胱抑素C单克隆抗体(包被) Alias Cystatin-3; Cystatin3; Cystatin 3; CystatinC; CST 3; CST3; CST-3; Cystatin C; AD 8; AD8; Amyloid angiopathy and cerebral hemorrhage; Cst 3; Cst3; CST3 protein; Gamma trace; HCCAA; Neuroendocrine basic polypeptide; Post gamma globulin; ARMD11; MGC117328; CYSC; CYTC_HUMAN. SLM-41184M Research Area Tumour Cardiovascular Cell biology immunology Immunogen Species Mouse Clonality Monoclonal Clone NO. 8F14 React Species (predicted: Human, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 13kDa Cellular localization Secretory protein Form Liquid Concentration 1mg/ml immunogen Recombinant human Cystatin-C Lsotype IgG Purification affinity purified by Protein G Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail SLM-41183M(capture) is Matched Pair with SLM-41184M(detection).
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.
SWISS:
P01034
Gene ID:
1471
Database links:Entrez Gene: 1471 Human
Entrez Gene: 13010 Mouse
Omim: 604312 Human
SwissProt: P01034 Human
SwissProt: P21460 Mouse
Unigene: 304682 Human
Unigene: 4263 Mouse
Unigene: 106351 Rat
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