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Working model for the function of Mvb12. In the cytosol, Mvb12 associates with and stabilizes ESCRT-I in an oligomeric, inactive state so that ESCRT-I cannot interact with ESCRT-II. Once recruited to the endosome, ESCRT-I assembles with ESCRT-II, leading to the activation of the downstream ESCRT machinery and cargo sorting into the MVB pathway. In the absence of Mvb12, the assembly of ESCRT-I and -II occurs prematurely in the cytosol, resulting in a defect in MVB sorting.
