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merck millipore,默克密理博,MAB1948P,Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6
产品名称：Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6
产品型号：MAB1948P
Detect Heparan Sulfate Proteoglycan (Perlecan) using this Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6 validated for use in IH, IC, WB & IP.
merck millipore,默克密理博,MAB1948P,Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB, IHC, ICC R Purified Monoclonal Antibody 描述 产品目录编号 MAB1948P Replaces MAB1948 描述 Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6 Alternate Names - heparan sulfate proteoglycan of basement membrane
- heparan sulfate proteoglycan 2
- Schwartz-Jampel syndrome 1 (chondrodystrophic myotonia)
- endorepellin (domain V region)
- perlecan proteoglycan
背景信息 Heparan sulfate proteoglycans (HSPGs) are found on cell surfaces and in the extracelluar matrix of all mammalian tissues, particularly in the core of the basement membrane. HSPGs found in the brain are dystroglycan, N-syndecan, glypican, and perlecan. The composition of HSPGs differs in the protein core, and is thought to determine the location of HSPG in the cell membrane (syndecan and glypican) and extracellular matrix (perlecan and dystroglycan). The postulated functions of HSPG include cell proliferation, differentiation, adhesion, migration, and morphogenesis.
Heparan Sulfate Proteoglycan antibodies, in tissues, react strongly and uniformly with basement membranes. Clone A7L6 recognizes domain IV of the core protein of the large heparan sulphate proteoglycan or perlecan. The reactivity is independent of the galactosaminoglycan moieties; therefore, the epitope is not sensitive to heparinase treatment.产品信息 格式 Purified 控制 - HeLa and A431 cells
演示 Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Detect Heparan Sulfate Proteoglycan (Perlecan) using this Anti-Heparan Sulfate Proteoglycan (Perlecan) Antibody, clone A7L6 validated for use in IH, IC, WB & IP. 主要应用 - Western Blotting
- Immunohistochemistry
- Immunocytochemistry
应用说明 Immunohistochemistry Analysis: 1:100 dilution from a previous lot detected Heparan Sulfate Proteoglycan in large cell carcinoma tissue.
Western Blot Analysis: A previous lot was used in independent laboratories in WB. (Hagen, 1993; Brown, 1999).
Immunoprecipitation Analysis: A previous lot was used by an independent laboratory in IP. (Couchman, 1989).生物信息 免疫原品种 Heparan Sulfate Proteoglycan from EHS mouse tumor 表位 HSPG core protein 克隆 A7L6 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rat 特异性 The antibody recognizes a high molecular weight core protein of Heparan Sulfate Proteoglycan (Perlecan). No cross reactivity to laminin, collagen IV, entactin/nidogen, or fibronectin (Horiguchi, 1989). 同种型 IgG2aκ 品种反应性 Human Species Reactivity Note Demonstrated to react with human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_005520
Entrez基因汇总 This gene encodes the perlecan protein, which consists of a core protein to which three long chains of glycosaminoglycans (heparan sulfate or chondroitin sulfate) are attached. The perlecan protein is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix components and cell-surface molecules. It has been shown that this protein interacts with laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and Transthyretin, etc. and plays essential roles in multiple biological activities. Perlecan is a key component of the vascular extracellular matrix, where it helps to maintain the endothelial barrier function. It is a potent inhibitor of smooth muscle cell proliferation and is thus thought to help maintain vascular homeostasis. It can also promote growth factor (e.g., FGF2) activity and thus stimulate endothelial growth and re-generation. It is a major component of basement membranes, where it is involved in the stabilization of other molecules as well as being involved with glomerular permeability to macromolecules and cell adhesion. Mutations in this gene cause Schwartz-Jampel syndrome type 1, Silverman-Handmaker type of dyssegmental dysplasia, and Tardive dyskinesia. 基因符号 - PLC
- HSPG2
- SJS
- PRCAN
- SJS1
- SJA
- perlecan
- HSPG
- Perlecan
纯化方法 Protein G purfied UniProt编号 - P98160
UniProt汇总 FUNCTION: This protein is an integral component of basement membranes. It is responsible for the fixed negative electrostatic charge and is involved in the charge-selective ultrafiltration properties. It serves as an attachment substrate for cells.
SIZE: 4391 amino acids; 468825 Da
SUBUNIT: Purified perlecan has a strong tendency to aggregate in dimers or stellate structures. It interacts with other basement membrane components such as laminin, prolargin and collagen type IV. Interacts with COL13A1, FGFBP1 and VWA1.
SUBCELLULAR LOCATION: Secreted, extracellular space.
TISSUE SPECIFICITY: Found in the basement membranes.
PTM: N- and O-glycosylated; contains three heparan sulfate chains.
DISEASE: Defects in HSPG2 are the cause of Schwartz-Jampel syndrome (SJS1) [MIM:255800]; a rare autosomal recessive disorder characterized by permanent myotonia (prolonged failure of muscle relaxation) and skeletal dysplasia, resulting in reduced stature, kyphoscoliosis, bowing of the diaphyses and irregular epiphyses & defects in HSPG2 are the cause of dyssegmental dysplasia Silverman-Handmaker type (DDSH) [MIM:224410]. The dyssegmental dysplasias are rare, autosomal recessive skeletal dysplasias with anisospondyly and micromelia. There are two recognized types: the severe, lethal DDSH and the milder Rolland-Desbuquois form. Individuals with DDSH also have a flat face, micrognathia, cleft palate and reduced joint mobility, and frequently have an encephalocoele. The endochondral growth plate is short, the calcospherites (which are spherical calcium-phosphorus crystals produced by hypertrophic chondrocytes) are unfused, and there is mucoid degeneration of the resting cartilage.
SIMILARITY: Contains 4 EGF-like domains. & Contains 22 Ig-like C2-type (immunoglobulin-like) domains. & Contains 11 laminin EGF-like domains. & Contains 3 laminin G-like domains. & Contains 3 laminin IV type A domains. & Contains 4 LDL-receptor class A domains. & Contains 1 SEA domain.产品使用声明 质量保证 Evaluated by Immunocytochemistry in HeLa and A431 cells.
Immunocytochemistry Analysis: 1:500 dilution of this antibody detected Heparan Sulfate Proteoglycan in HeLa and A431 cells.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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