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Worsening of Huntington disease phenotype in CB1 receptor knockout mice.
Neurobiol Dis 2011; 42(3):524-9ND

Abstract

Huntington's disease (HD) is a progressive neurodegenerative genetic disorder which leads to motor, cognitive and psychiatric disturbances. The primary neuropathological hallmark is atrophy of the striatum. Cannabinoid CB1 receptors (CB1Rs) are particularly enriched in the striatum and previous works indicate their early loss of expression in HD, even before symptom occurrence. However, pathophysiological significance of this loss of expression remains unclear. In addition, whether specific modulation of CB1R is able to mitigate striatal neuron fate in HD remains currently controversial. In order to gain further insights on the potential role of CB1R in HD physiopathology, we evaluated the pathophysiological consequences of a genetic deletion of CB1R in the N171-82Q transgenic model and following 3-nitropropionic (3NP) intoxication. Taken together our data demonstrate that CB1R knockout (1) worsens motor performances in N171-82Q mice and (2) increases mouse susceptibility to 3NP. These results suggest that functional changes in CB1R may contribute to the physiopathological development of HD.

Authors+Show Affiliations

IRIBHM, Université Libre de Bruxelles, Brussels, Belgium.No affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

21406230

Citation

Mievis, Stéphane, et al. "Worsening of Huntington Disease Phenotype in CB1 Receptor Knockout Mice." Neurobiology of Disease, vol. 42, no. 3, 2011, pp. 524-9.
Mievis S, Blum D, Ledent C. Worsening of Huntington disease phenotype in CB1 receptor knockout mice. Neurobiol Dis. 2011;42(3):524-9.
Mievis, S., Blum, D., & Ledent, C. (2011). Worsening of Huntington disease phenotype in CB1 receptor knockout mice. Neurobiology of Disease, 42(3), pp. 524-9. doi:10.1016/j.nbd.2011.03.006.
Mievis S, Blum D, Ledent C. Worsening of Huntington Disease Phenotype in CB1 Receptor Knockout Mice. Neurobiol Dis. 2011;42(3):524-9. PubMed PMID: 21406230.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Worsening of Huntington disease phenotype in CB1 receptor knockout mice. AU - Mievis,Stéphane, AU - Blum,David, AU - Ledent,Catherine, Y1 - 2011/03/22/ PY - 2010/11/04/received PY - 2011/02/15/revised PY - 2011/03/06/accepted PY - 2011/3/17/entrez PY - 2011/3/17/pubmed PY - 2011/8/24/medline SP - 524 EP - 9 JF - Neurobiology of disease JO - Neurobiol. Dis. VL - 42 IS - 3 N2 - Huntington's disease (HD) is a progressive neurodegenerative genetic disorder which leads to motor, cognitive and psychiatric disturbances. The primary neuropathological hallmark is atrophy of the striatum. Cannabinoid CB1 receptors (CB1Rs) are particularly enriched in the striatum and previous works indicate their early loss of expression in HD, even before symptom occurrence. However, pathophysiological significance of this loss of expression remains unclear. In addition, whether specific modulation of CB1R is able to mitigate striatal neuron fate in HD remains currently controversial. In order to gain further insights on the potential role of CB1R in HD physiopathology, we evaluated the pathophysiological consequences of a genetic deletion of CB1R in the N171-82Q transgenic model and following 3-nitropropionic (3NP) intoxication. Taken together our data demonstrate that CB1R knockout (1) worsens motor performances in N171-82Q mice and (2) increases mouse susceptibility to 3NP. These results suggest that functional changes in CB1R may contribute to the physiopathological development of HD. SN - 1095-953X UR - https://www.unboundmedicine.com/medline/citation/21406230/abstract/Worsening_of_Huntington_dise L2 - https://linkinghub.elsevier.com/retrieve/pii/S0969-9961(11)00088-X DB - PRIME DP - Unbound Medicine ER -