Anti-CST3 Antibody (MACO0542)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- MACO0542
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Mouse
- Isotype:
- IgG2b
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Monoclonal Antibody
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Product Name: | Anti-CST3 Antibody |
Product Sku: | MACO0542 |
Size: | 50ug |
Host Species: | Mouse |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | IHC:1:50-1:500 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Cystatin C protein |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
Purification Method: | >95%,Protein G purified |
Clonality: | Monoclonal |
Isotype: | IgG2b |
Conjugate: | Non-conjugated |
Background: | 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.Genetic variations in CST3 are associated with age-related macular degeneration type 11 (ARMD11) [MIM:611953]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. |
Synonyms: | CysC,Cystatin-3,Gamma-trace,Neuroendocrine basic polypeptide,Post-gamma-globulin |
Immunohistochemical of paraffin-embedded human kidney tissue using MACO0542 at dilution of 1:200 | |
Immunohistochemical of paraffin-embedded human prostate tissue using MACO0542 at dilution of 1:200 |
UniProt Protein Function: | CST3: As an inhibitor of cysteine proteinases, this protein is thought to serve an important physiological role as a local regulator of this enzyme activity. Defects in CST3 are the cause of amyloidosis type 6 (AMYL6); 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. Genetic variations in CST3 are associated with age- related macular degeneration type 11 (ARMD11). ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Belongs to the cystatin family. |
UniProt Protein Details: | Protein type:Secreted, signal peptide; Inhibitor; Secreted Chromosomal Location of Human Ortholog: 20p11.21 Cellular Component: extracellular space; extracellular region Molecular Function:protein binding; protease binding; beta-amyloid binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity Biological Process: negative regulation of proteolysis; fibril organization and biogenesis; negative regulation of peptidase activity; defense response; regulation of tissue remodeling Disease: Macular Degeneration, Age-related, 11; Cerebral Amyloid Angiopathy, Cst3-related |
NCBI Summary: | 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. In addition, this protein has been shown to have an antimicrobial function, inhibiting the replication of herpes simplex virus. Alternative splicing results in multiple transcript variants encoding a single protein. [provided by RefSeq, Nov 2014] |
UniProt Code: | P01034 |
NCBI GenInfo Identifier: | 118183 |
NCBI Gene ID: | 1471 |
NCBI Accession: | P01034.1 |
UniProt Related Accession: | P01034 |
Molecular Weight: | |
NCBI Full Name: | Cystatin-C |
NCBI Synonym Full Names: | cystatin C |
NCBI Official Symbol: | CST3Â Â |
NCBI Official Synonym Symbols: | ARMD11; HEL-S-2Â Â |
NCBI Protein Information: | cystatin-C |
UniProt Protein Name: | Cystatin-C |
UniProt Synonym Protein Names: | Cystatin-3; Gamma-trace; Neuroendocrine basic polypeptide; Post-gamma-globulin |
Protein Family: | Cystatin |
UniProt Gene Name: | CST3Â Â |
UniProt Entry Name: | CYTC_HUMAN |