An orally active anti-apoptotic molecule (CGP 3466B) preserves mitochondria and enhances survival in an animal model of motoneuron disease. Export

@article{citeulike:3206108, abstract = {Apoptosis and mitochondrial dysfunction are thought to be involved in the aetiology of neurodegenerative diseases. We have tested an orally active anti-apoptotic molecule (CGP 3466B) that binds to glyceraldehyde-3-phosphate dehydrogenase (GAPDH) in an animal model with motoneuron degeneration, i.e. a mouse mutant with progressive motor neuronopathy (pmn). In pmn/pmn mice, CGP 3466B was administered orally (10 - 100 nmol kg(-1)) at the onset of the clinical symptoms (2 weeks after birth). CGP 3466B slowed disease progression as determined by a 57\% increase in life-span, preservation of body weight and motor performance. This improvement was accompanied by a decreased loss of motoneurons and motoneuron fibres as well as an increase in retrograde transport. Electron microscopic analysis showed that CGP 3466B protects mitochondria which appear to be selectively disrupted in the motoneurons of pmn/pmn mice. The data support evaluation of CGP 3466B as a potential treatment for motor neuron disease.}, address = {Department of APSIC and Division of Clinical Neuromuscular Research, Faculty of Medicine, Geneva University, 1211 Geneva 4, Switzerland.}, author = {Sagot, Y. and Toni, N. and Perrelet, D. and Lurot, S. and King, B. and Rixner, H. and Mattenberger, L. and Waldmeier, P. C. and Kato, A. C.}, citeulike-article-id = {3206108}, citeulike-linkout-0 = {http://dx.doi.org/10.1038/sj.bjp.0703633}, citeulike-linkout-1 = {http://view.ncbi.nlm.nih.gov/pubmed/11030721}, citeulike-linkout-2 = {http://www.hubmed.org/display.cgi?uids=11030721}, doi = {10.1038/sj.bjp.0703633}, issn = {0007-1188}, journal = {British journal of pharmacology}, keywords = {als, apoptosis, therapy}, month = {October}, number = {4}, pages = {721--728}, posted-at = {2008-09-08 13:44:36}, priority = {1}, title = {An orally active anti-apoptotic molecule (CGP 3466B) preserves mitochondria and enhances survival in an animal model of motoneuron disease.}, url = {http://dx.doi.org/10.1038/sj.bjp.0703633}, volume = {131}, year = {2000} }

TY - JOUR ID - citeulike:3206108 L3 - citeulike-article-id:3206108 N2 - Apoptosis and mitochondrial dysfunction are thought to be involved in the aetiology of neurodegenerative diseases. We have tested an orally active anti-apoptotic molecule (CGP 3466B) that binds to glyceraldehyde-3-phosphate dehydrogenase (GAPDH) in an animal model with motoneuron degeneration, i.e. a mouse mutant with progressive motor neuronopathy (pmn). In pmn/pmn mice, CGP 3466B was administered orally (10 - 100 nmol kg(-1)) at the onset of the clinical symptoms (2 weeks after birth). CGP 3466B slowed disease progression as determined by a 57% increase in life-span, preservation of body weight and motor performance. This improvement was accompanied by a decreased loss of motoneurons and motoneuron fibres as well as an increase in retrograde transport. Electron microscopic analysis showed that CGP 3466B protects mitochondria which appear to be selectively disrupted in the motoneurons of pmn/pmn mice. The data support evaluation of CGP 3466B as a potential treatment for motor neuron disease. IS - 4 JF - British journal of pharmacology SN - 0007-1188 AD - Department of APSIC and Division of Clinical Neuromuscular Research, Faculty of Medicine, Geneva University, 1211 Geneva 4, Switzerland. EP - 728 TI - An orally active anti-apoptotic molecule (CGP 3466B) preserves mitochondria and enhances survival in an animal model of motoneuron disease. VL - 131 SP - 721 KW - als KW - apoptosis KW - therapy AU - Sagot, Y AU - Toni, N AU - Perrelet, D AU - Lurot, S AU - King, B AU - Rixner, H AU - Mattenberger, L AU - Waldmeier, PC AU - Kato, AC PY - 2000/10// UR - http://dx.doi.org/10.1038/sj.bjp.0703633 ER -