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Rabbit Anti-WISP3/BF350 Conjugated antibody (bs-12380R-BF350)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-12380R-BF350
英文名称 Rabbit Anti-WISP3/BF350 Conjugated antibody
中文名称 BF350标记的Wnt1信号通路蛋白3抗体
别    名 CCN 6; CCN family member 6; CCN6; CYR61; LIBC; Lost in inflammatory breast cancer tumor suppressor protein; MGC125987; MGC125988; MGC125989; OTTHUMP00000040421; PPAC; PPD; UNQ462/PRO790/PRO956; WISP 3; WISP-3; WISP3; WISP3_HUMAN; WNT 1 inducible signaling pathway protein 3; Wnt 1 signaling pathway protein 3; WNT1 inducible signaling pathway protein 3; WNT1 inducible signaling pathway protein 3 precursor; WNT1-inducible-signaling pathway protein 3.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  发育生物学  信号转导  干细胞  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Dog, Horse, Rabbit, Sheep, Monkey, )
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 37kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WISP3
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:
Wnt-induced secreted protein (WISP)-1, WISP-2 and WISP-3 are members of the CCN family of growth factors, which include connective tissue growth factor (CTGF) and Cyr61. WISP-1, WISP-2 and WISP-3 share significant sequence similarity, including four conserved cysteine-rich domains, and they are believed to function as dimers in their active forms. WISP-1 expression is observed in various tissues including adult heart, kidney and spleen, while WISP-2 expression predominates in skeletal muscle, colon and ovary. Both WISP-1 and WISP-2 are upregulated in cells transformed with the proto-oncogene Wnt-1, and they are also more highly expressed in human colon tumors, suggesting that these proteins may participate in tumor development. WISP-3 is involved in normal post-natal skeletal growth, and it is also implicated in the development of the autosomal recessive skeletal disorder progressive pseudorheumatoid dysplasia, which affects cartilage homeostasis by disrupting the growth of chondrocyte and normal cell columnar organization.

Function:
Appears to be required for normal postnatal skeletal growth and cartilage homeostasis.

Subcellular Location:
Secreted.

Tissue Specificity:
Predominant expression in adult kidney and testis and fetal kidney. Weaker expression found in placenta, ovary, prostate and small intestine. Also expressed in skeletally-derived cells such as synoviocytes and articular cartilage chondrocytes.

DISEASE:
Defects in WISP3 are the cause of progressive pseudorheumatoid arthropathy of childhood (PPAC) [MIM:208230]. PPAC is an autosomal recessive disorder characterized by stiffness and swelling of joints, motor weakness and joint contractures. Signs and symptoms of the disease develop typically between three and eight years of age. This progressive disease is a primary disorder of articular cartilage with continued cartilage loss and destructive bone changes with aging.

Similarity:
Belongs to the CCN family.
Contains 1 CTCK (C-terminal cystine knot-like) domain.
Contains 1 IGFBP N-terminal domain.
Contains 1 TSP type-1 domain.

Database links:

Entrez Gene: 8838 Human

Entrez Gene: 327743 Mouse

Entrez Gene: 499461 Rat

Omim: 603400 Human

SwissProt: O95389 Human

SwissProt: D3Z5L9 Mouse

Unigene: 558428 Human

Unigene: 208111 Mouse

Unigene: 141375 Rat



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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