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To avoid massive overproduction of proteins in women that could potentially be lethal, the female body has a unique mechanism for shutting down protein production in one of the XX pair

Strub, M.-P

These class II CFTR variants are associated with altered structural conformation, and this misfolded CFTR proteins is degraded by the chaperons mediated Endoplasmic Reticulum-Associated Degradation (ERAD) pathway

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