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WISP3 Rabbit pAb (bs-12380R)  
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產(chǎn)品編號 bs-12380R
英文名稱 WISP3 Rabbit pAb
中文名稱 Wnt1信號通路蛋白3抗體
別    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.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  發(fā)育生物學(xué)  信號轉(zhuǎn)導(dǎo)  干細(xì)胞  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應(yīng) Rat (predicted: Human,Mouse,Rabbit,Sheep,Dog,Horse,Monkey)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 37 kDa
檢測分子量
細(xì)胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WISP3: 221-320/354 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
O95389

Gene ID:
8838

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



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (WISP3) Polyclonal Antibody, Unconjugated (bs-12380R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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