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Review Article
Mechanisms of Disease
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Volume 343:1095-1104 October 12, 2000 Number 15
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Genetic Defects of Intracellular-Membrane Transport
Vesa M. Olkkonen, Ph.D., and Elina Ikonen, M.D., Ph.D.

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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 . . . [Full Text of this Article]

Intracellular-Membrane Transport

Sorting of Proteins to Transport Vesicles

Cytoskeletal Tracks for the Movement of Vesicles

Fusion of Transport Vesicles with Target Membranes

Inherited Diseases Characterized by Defects in Intracellular Protein Transport

Defects Affecting the Protein-Sorting Apparatus

Defects in Cytoskeletal Proteins Involved in Membrane Transport

Defects in the Machinery for the Docking and Fusion of Transport Vesicles

Lipid Disorders Affecting Vesicular Transport

Conclusions


Source Information

From the Department of Biochemistry, National Public Health Institute, Helsinki, Finland.

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