Efficacy of exogenous pyruvate in Trembler^J mouse model of Charcot-Marie-Tooth neuropathy.

ABSTRACT:

Introduction

Classic Charcot-Marie-Tooth (CMT) neuropathies including those with Schwann cell genetic defects exhibit a length-dependent process affecting the distal axon. Energy deprivation in the distal axon has been the proposed mechanism accounting for length-dependent distal axonal degeneration. We hypothesized that pyruvate, an intermediate glycolytic product, could restore nerve function, supplying lost energy to the distal axon.

Methods

To test this possibility, we supplied pyruvate to the drinking water of the Trembler-J (Tr^J ) mouse and assessed efficacy based on histology, electrophysiology, and functional outcomes. Pyruvate outcomes were compared with untreated Tr^J controls alone or adeno-associated virus mediated NT-3 gene therapy (AAV1.NT-

SUBMITTER: Sahenk Z 

PROVIDER: S-EPMC6192403 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature