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Human Molecular Genetics Advance Access first published online on May 11, 2005
This version published online on May 25, 2005
Human Molecular Genetics, doi:10.1093/hmg/ddi189
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© The Author 2005. Published by Oxford University Press. All rights reserved
Received January 17, 2005
Accepted May 4, 2005

Article

TREATMENT WITH TRK C AGONIST ANTIBODIES DELAYS DISEASE PROGRESSION IN NEUROMUSCULAR DEGENERATION (nmd) MICE

Rocio Ruiz 1, John Lin 2, Alison Forgie 2, Davide Foletti 2, David Shelton 2, Arnon Rosenthal 2, and Lucia Tabares 1*

1 Dept. of Physiology & Biophysics. School of Medicine. University of Seville. Spain.
2 Rinat Neuroscience Corp. 3155 Porter Drive. Palo Alto, CA 94304. USA

* To whom correspondence should be addressed.
Lucia Tabares, E-mail: ltabares{at}us.es


  Abstract

Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a fatal autosomal recessive disorder of infants. It is characterized by lower motor neuron degeneration, progressive muscle paralysis and respiratory failure, for which no effective treatment exists. The phenotype of nmd (neuromuscular degeneration) mice closely resembles the human SMARD1. The identification of the mutated mouse gene in nmd mice, Ighmbp2, led to the discovery of mutations of the homologous gene in humans with SMARD1. We have studied the nmd mouse model with in vivo electrophysiological techniques and evaluated the efficacy of Mab2256, a monoclonal antibody with agonist effect on the tyrosine kinase receptor C, trkC, on disease progression in nmd mice. Treatment with Mab2256 resulted in a significant but transient improvement of muscle strength in nmd mice, as well as normalization of the neuromuscular depression during high frequency nerve stimulation. These results suggest the potential of using monoclonal agonist antibodies for neurotrophin receptors in lower motor neuron diseases such as SMARD1.

The originally published versions 1 and 2 of this paper were not the right ones and so have been replaced with the correct version.
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