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Rabbit Anti-Lubricin/FITC Conjugated antibody (bs-11175R-FITC)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11175R-FITC
英文名稱 Rabbit Anti-Lubricin/FITC Conjugated antibody
中文名稱 FITC標記的巨核細胞刺激因子/蛋白多糖4/淺表層粘膜蛋白多糖抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 信號轉(zhuǎn)導(dǎo)  細胞骨架  細胞外基質(zhì)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Dog, Cow, Rabbit, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Lubricin/SZP
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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(chǎn)品介紹 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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