|Other Names||Cardiotrophin-like cytokine factor 1, B-cell-stimulating factor 3, BSF-3, Novel neurotrophin-1, NNT-1, CLCF1, BSF3, CLC, NNT1|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||BSF3, CLC, NNT1|
|Function||Cytokine with B-cell stimulating capability. Binds to and activates the ILST/gp130 receptor.|
|Tissue Location||Expressed predominantly in lymph nodes, spleen, peripheral blood lymphocytes, bone marrow, and fetal liver.|
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This gene is a member of the glycoprotein (gp)130 cytokinefamily and encodes cardiotrophin-like cytokine factor 1 (CLCF1).CLCF1 forms a heterodimer complex with cytokine receptor-likefactor 1 (CRLF1). This dimer competes with ciliary neurotrophicfactor (CNTF) for binding to the ciliary neurotrophic factorreceptor (CNTFR) complex, and activates the Jak-STAT signalingcascade. CLCF1 can be actively secreted from cells by forming acomplex with soluble type I CRLF1 or soluble CNTFR. CLCF1 is apotent neurotrophic factor, B-cell stimulatory agent andneuroendocrine modulator of pituitary corticotroph function.Defects in CLCF1 cause cold-induced sweating syndrome 2 (CISS2).This syndrome is characterized by a profuse sweating after exposureto cold as well as congenital physical abnormalities of the headand spine. Alternative splicing results in multiple transcriptvariants encoding distinct isoforms.
Rousseau, F., et al. J. Biol. Chem. 283(44):30341-30350(2008)Dagoneau, N., et al. Am. J. Hum. Genet. 80(5):966-970(2007)Rousseau, F., et al. Proc. Natl. Acad. Sci. U.S.A. 103(26):10068-10073(2006)Perret, D., et al. J. Biol. Chem. 279(42):43961-43970(2004)Burger, R., et al. Br. J. Haematol. 123(5):869-878(2003)
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