FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体

FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体

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产品名称: FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体

英文名称: Anti-Lubricin/FITC

产品编号: HZ-11175R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-Lubricin/FITC Conjugated antibody

FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体

 

产品编号 bs-11175R-FITC
英文名称 Anti-Lubricin/FITC
中文名称 FITC标记的巨核细胞刺激因子/蛋白多糖4/浅表层粘膜蛋白多糖抗体
别    名 Superficial zone proteoglycan; articular superficial zone protein; bG174L6.2; CACP; camptodactyly arthropathy coxa vara pericarditis syndrome gene; FLJ32635; HAPO; Jacobs camptodactyly-arthropathy-pericarditis syndrome gene; JCAP; megakaryocyte stimulating factor; MSF; PRG 4; PRG4; proteoglycan 4; Proteoglycan4; Superficial zone proteoglycan; SZP; PRG4_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 信号转导  细胞骨架  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Rabbit, 
产品应用 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 152kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Lubricin/SZP
亚    型 IgG
纯化方法 affinity purified by Protein A
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.
产品介绍 background:
Lubricin, also designated proteoglycan-4 or megakaryocyte stimulating factor, is important for boundary lubrication within articulating joints. It is a disulfide-linked homodimer (between Cysteine 1146 and Cysteine 1403) that is essential for protein cleavage. Lubricin inhibits synovial cell adhesion to the cartilage surface, but also prevents the deposition of proteins from synovial fluid onto cartilage. Lubricin is highly expressed in cartilage, liver and synovial tissue. Defects in the gene encoding for lubricin can cause Jakobs syndrome, also designated camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP). CACP is an autosomal recessive disorder characterized by joint failure associated with noninflammatory synoviocyte hyperplasia and subinitimal fibrosis of the synovial capsule. Lubricin undergoes different levels of glycosylation and may be detected at varying molecular weights.

Function:
Plays a role in boundary lubrication within articulating joints. Prevents protein deposition onto cartilage from synovial fluid by controlling adhesion-dependent synovial growth and inhibiting the adhesion of synovial cells to the cartilage surface. 
Isoform F plays a role as a growth factor acting on the primitive cells of both hematopoietic and endothelial cell lineages. soluble molecule that acts as a carrier for insoluble surface-active phospholipid (SAPL). Depletion of lubricin function has been associated with camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP), an arthritic-like autosomal recessive disorder.

Subunit:
Homodimer; disulfide-linked.

Subcellular Location:
Secreted.

Tissue Specificity:
Highly expressed in synovial tissue, cartilage and liver and weakly in heart and lung. Isoform B is expressed in kidney, lung, liver, heart and brain. Isoform C and isoform D are widely expressed.

Post-translational modifications:
N-glycosylated. 
O-glycosylated; contains glycosaminoglycan chondroitin sulfate and keratan sulfate. 
The disulfide bond between Cys-1146 and Cys-1403 is essential for protein cleavage. 

DISEASE:
Defects in PRG4 are the cause of camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) [MIM:208250]; also known as Jacobs syndrome. CACP is an autosomal recessive disorder. Individuals with CACP have normal appearing joints at birth but with advancing age develop joint failure associated with noninflammatory synoviocyte hyperplasia and subintimal fibrosis of the synovial capsule.

Similarity:
Contains 2 hemopexin-like domains.
Contains 2 SMB (somatomedin-B) domains. 

Database links:

Entrez Gene: 10216 Human

Entrez Gene: 280867 Cow

Entrez Gene: 96875 Mouse

Omim: 604283 Human

SwissProt: Q92954 Human

SwissProt: Q9JM99 Mouse

Unigene: 647723 Human

Unigene: 329131 Mouse



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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润滑剂,也被指定为蛋白聚糖-4或巨核细胞刺激因子,对于关节关节内的边界润滑是重要的。它是二硫键连接的同型二聚体(半胱氨酸1146和半胱氨酸1403之间),这是蛋白质裂解必不可少的。润滑剂抑制滑膜细胞粘附到软骨表面,但也防止滑膜液中的蛋白质沉积到软骨上。润滑脂在软骨、肝脏和滑膜组织中高表达。编码润滑脂基因的缺陷可导致JAKOBOS综合征,也被称为叉指关节病-髋内翻性心包炎综合征(CACP)。CACP是一种常染色体隐性遗传疾病,其特征是关节炎与滑膜囊的非炎性滑膜细胞增生和亚显微纤维化有关。润滑剂经历不同水平的糖基化,并且可以在不同分子量下检测。