Genetic Defects of Intracellular-Membrane Transport

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Abstract
The compartmentalization of functions into distinct membrane-bound organelles is a central characteristic of cells. The protein and lipid composition of these organelles is unique, a factor that is vital for their proper function. This necessitates tightly controlled transport of biomolecules from their sites of synthesis or uptake to specific destinations, and mechanisms that prevent promiscuous interactions between cellular membranes that would lead to deleterious mixing of organelle constituents. One of the major processes responsible for the correct localization of molecules within the cell is called membrane or vesicular transport. In this process, membranous carrier structures bud off a donor compartment . . .