Transport of Trembler-J Mutant Peripheral Myelin Protein 22 Is Blocked in the Intermediate Compartment and Affects the Transport of the Wild-Type Protein by Direct Interaction

Peripheral myelin protein 22 (PMP22) is an integral membrane protein that is essential for the normal formation and maintenance of peripheral myelin. Duplications, deletions, or mutations in the PMP22 gene account for a set of dominantly inherited peripheral neuropathies. The heterozygous Trembler-J...

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Veröffentlicht in:	The Journal of neuroscience 1999-03, Vol.19 (6), p.2027-2036
Hauptverfasser:	Tobler, Andreas R, Notterpek, Lucia, Naef, Roland, Taylor, Verdon, Suter, Ueli, Shooter, Eric M
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biological Transport - physiology COS Cells Dimerization Epitopes - physiology Mice Mice, Neurologic Mutants - metabolism Mutation - physiology Myelin Proteins - chemistry Myelin Proteins - genetics Myelin Proteins - immunology Myelin Proteins - metabolism Reference Values Schwann Cells - metabolism
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