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Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid.
Exp Neurol. 2001 Apr; 168(2):356-63.EN

Abstract

The pathogenesis of neurodegenerative diseases may involve a genetic predisposition acting in concert with environmental toxins. To test this hypothesis we examined whether transgenic mice with the G93A mutation in Cu,Zn superoxide dismutase show increased vulnerability to either 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) or 3-nitropropionic acid (3-NP). Compared to littermate controls G93A transgenic mice showed a greater loss of striatal dopamine, DOPAC, and HVA at 50, 70, and 120 days of age following administration of MPTP; however, cell loss in the substantia nigra was not greater. The G93A transgenic mice showed significantly increased vulnerability to striatal lesions produced by 3-NP compared with littermate controls at 120 days of age. The finding that G93A mice show increased vulnerability to mitochondrial toxins further implicates mitochondrial dysfunction in the pathogenesis of neuronal death in these mice. The findings support the hypothesis that a genetic defect can increase susceptibility to environmental toxins and that this may play a role in the pathogenesis of neurodegenerative diseases.

Authors+Show Affiliations

Neurochemistry Laboratory, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

11259123

Citation

Andreassen, O A., et al. "Transgenic ALS Mice Show Increased Vulnerability to the Mitochondrial Toxins MPTP and 3-nitropropionic Acid." Experimental Neurology, vol. 168, no. 2, 2001, pp. 356-63.
Andreassen OA, Ferrante RJ, Klivenyi P, et al. Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid. Exp Neurol. 2001;168(2):356-63.
Andreassen, O. A., Ferrante, R. J., Klivenyi, P., Klein, A. M., Dedeoglu, A., Albers, D. S., Kowall, N. W., & Beal, M. F. (2001). Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid. Experimental Neurology, 168(2), 356-63.
Andreassen OA, et al. Transgenic ALS Mice Show Increased Vulnerability to the Mitochondrial Toxins MPTP and 3-nitropropionic Acid. Exp Neurol. 2001;168(2):356-63. PubMed PMID: 11259123.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid. AU - Andreassen,O A, AU - Ferrante,R J, AU - Klivenyi,P, AU - Klein,A M, AU - Dedeoglu,A, AU - Albers,D S, AU - Kowall,N W, AU - Beal,M F, PY - 2001/3/22/pubmed PY - 2001/5/22/medline PY - 2001/3/22/entrez SP - 356 EP - 63 JF - Experimental neurology JO - Exp Neurol VL - 168 IS - 2 N2 - The pathogenesis of neurodegenerative diseases may involve a genetic predisposition acting in concert with environmental toxins. To test this hypothesis we examined whether transgenic mice with the G93A mutation in Cu,Zn superoxide dismutase show increased vulnerability to either 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) or 3-nitropropionic acid (3-NP). Compared to littermate controls G93A transgenic mice showed a greater loss of striatal dopamine, DOPAC, and HVA at 50, 70, and 120 days of age following administration of MPTP; however, cell loss in the substantia nigra was not greater. The G93A transgenic mice showed significantly increased vulnerability to striatal lesions produced by 3-NP compared with littermate controls at 120 days of age. The finding that G93A mice show increased vulnerability to mitochondrial toxins further implicates mitochondrial dysfunction in the pathogenesis of neuronal death in these mice. The findings support the hypothesis that a genetic defect can increase susceptibility to environmental toxins and that this may play a role in the pathogenesis of neurodegenerative diseases. SN - 0014-4886 UR - https://www.unboundmedicine.com/medline/citation/11259123/Transgenic_ALS_mice_show_increased_vulnerability_to_the_mitochondrial_toxins_MPTP_and_3_nitropropionic_acid_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0014-4886(01)97627-7 DB - PRIME DP - Unbound Medicine ER -