New Vistas on the Pathomechanism of Charcot-Marie-Tooth and Related Peripheral Neuropathies

Annals of the New York Academy of Sciences
H W Müller

Abstract

A gene-dosage mechanism in CMT1A and HNPP has been postulated previously. Here, recent findings are discussed concerning (i) the functional consequences of altered PMP22 expression on Schwann cell growth regulation and on the capacity of genetically modified Schwann cells to myelinate peripheral axons, (ii) the cell physiological effects caused by the expression of certain disease-related missense mutations of PMP22 that are known to alter the Schwann cell phenotype and impair myelination in vivo, and (iii) the pathomechanism of CMT1 in light of findings on a novel association between PMP22 and P0 in PNS myelin.

References

May 15, 1992·Proceedings of the National Academy of Sciences of the United States of America·U SuterE M Shooter
Mar 19, 1992·Nature·U SuterE M Shooter
Jun 1, 1990·Molecular and Cellular Biology·G ManfiolettiC Schneider
Apr 1, 1994·Trends in Genetics : TIG·P I Patel, J R Lupski
Aug 15, 1994·Journal of Neuroscience Research·K A Nave
Apr 1, 1994·Journal of Neuroscience Research·C O HanemannH W Müller
Jan 1, 1995·Developmental Neuroscience·K Ikenaka, T Kagawa
Nov 1, 1995·Trends in Biochemical Sciences·P J ThomasP L Pedersen
May 1, 1996·Neuron·M SeredaK A Nave
Jun 1, 1996·Annals of Neurology·J M VallatA Brice
Feb 1, 1996·Current Opinion in Neurobiology·R Mirsky, K R Jessen
Oct 1, 1996·Brain : a Journal of Neurology·C O HanemannG Stoll
Feb 1, 1997·Trends in Biochemical Sciences·H L Pahl, P A Baeuerle
Apr 1, 1997·Journal of Neuroscience Research·D D'UrsoH W Müller
Sep 25, 1997·Journal of Neuroscience Research·D D'Urso, H W Müller
Jan 7, 1998·Journal of Anatomy·R Martini
Jul 31, 1998·Trends in Neurosciences·C O Hanemann, H W Müller
Feb 11, 1999·Journal of Neuroscience Research·F BosseH W Müller
Jul 9, 1999·Molecular Biology of the Cell·C BrancoliniC Schneider

❮ Previous
Next ❯

Citations


❮ Previous
Next ❯

Related Concepts

Related Feeds

Brain developing: Influences & Outcomes

This feed focuses on influences that affect the developing brain including genetics, fetal development, prenatal care, and gene-environment interactions. Here is the latest research in this field.