This study unveils the dynamic partnership between two key myelin proteins, PMP22 and MPZ, which are both critical for healthy peripheral nerve function. These abundant proteins in Schwann cells, form a specialized complex that likely holds myelin wraps together, ensuring rapid electrical signal transmission. Their interaction is important because disruption of either protein leads to debilitating conditions like Charcot-Marie-Tooth disease. Notably, we find that the A67T disease variant of PMP22 specifically disrupts this complex and causing a loss of PMP22 function and a peripheral neuropathy phenotype. What the association of PMP22 with MPZ provides at the cellular and molecular levels remains to be determined in our future studies.
Adhesion partners for compact myelin
robatlysosome
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