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However, other PDZ-binding proteins are known to also regulate CFTR localization and function, including NHERF2 55,84 , CAL (CFTR-associated ligand) 85 , and CAP70 (CFTR associated protein) 86 Furthermore, other non-PDZ-dependent mechanisms may contribute, since the physiologic dimers of CFTR are assembled independently of either NHERF1 or NHERF2, thus leaving PDZ-domains available for other interactions 55

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This strict protocol is critical because globally, 10-15% of untreated cases lead to neuropathy within just six months

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