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Family of CNTF-receptor–pathway related disorders. A, Phenotypic overlap of Crisponi syndrome, CISS1, CISS2, and SWS/SJS2. B, CNTF-receptor complex, composed of gp130, LIFRβ, and CNTFRα, required for binding CRLF1/CLCF1, which activates downstream signal–transduction events, including phosphorylation of STAT3. Black lines link each protein to the corresponding related disorder.1–8,20–22 (See also the London Medical Databases Web site.)
