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Neuroscience. 2017 Sep 1;358:261-268. doi: 10.1016/j.neuroscience.2017.06.061. Epub 2017 Jul 4.

Reduced axonal diameter of peripheral nerve fibers in a mouse model of Rett syndrome.

Author information

1
Institute of Neuroscience and Psychology, University of Glasgow, United Kingdom; Histology Department, Faculty of Medicine, Tanta University, Egypt.
2
Institute of Neuroscience and Psychology, University of Glasgow, United Kingdom; Pharmacology Department, Faculty of Medicine, Tanta University, Egypt.
3
Institute of Infection, Immunity and Inflammation College of Medical, Veterinary and Life Sciences, University of Glasgow, United Kingdom.
4
School of Life Sciences, College of Medical, Veterinary and Life Sciences, University of Glasgow, United Kingdom.
5
Institute of Neuroscience and Psychology, University of Glasgow, United Kingdom. Electronic address: stuart.cobb@glasgow.ac.uk.

Abstract

Rett syndrome (RTT) is a neurological disorder characterized by motor and cognitive impairment, autonomic dysfunction and a loss of purposeful hand skills. In the majority of cases, typical RTT is caused by de novo mutations in the X-linked gene, MECP2. Alterations in the structure and function of neurons within the central nervous system of RTT patients and Mecp2-null mouse models are well established. In contrast, few studies have investigated the effects of MeCP2-deficiency on peripheral nerves. In this study, we conducted detailed morphometric as well as functional analysis of the sciatic nerves of symptomatic adult female Mecp2+/- mice. We observed a significant reduction in the mean diameter of myelinated nerve fibers in Mecp2+/- mice. In myelinated fibers, mitochondrial densities per unit area of axoplasm were significantly altered in Mecp2+/- mice. However, conduction properties of the sciatic nerve of Mecp2 knockout mice were not different from control. These subtle changes in myelinated peripheral nerve fibers in heterozygous Mecp2 knockout mice could potentially explain some RTT phenotypes.

KEYWORDS:

MECP2; Rett syndrome; mitochondria; sciatic nerve

[Indexed for MEDLINE]

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