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Hence, cysteine modifications are not limited to the well-known structural role of disulfide bonds in proteins synthesized in the endoplasmic reticulum (ER), but participate in fundamental intra- and inter-cellular signaling pathways

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16,22-24 Tezacaftor/ivacaftor is approved for individuals aged 6 years and older with a homozygous F508del mutation or one tezacaftor/ivacaftor-responsive CFTR mutation