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Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease.
Neurobiol Dis. 2007 May; 26(2):375-84.ND

Abstract

Huntington's disease (HD) is a devastating neurodegenerative disease characterized by the selective loss of neurons in the striatum and cerebral cortex. This study tested the hypothesis that an adenoassociated viral (AAV2) vector encoding for the trophic factor neurturin (NTN) could provide neuroprotection in the rat 3-nitropropionic acid (3NP) model of HD. Rats received AAV2-NTN (CERE-120), AAV2-eGFP or Vehicle, followed 4 weeks later by the mitochondrial toxin 3NP. 3NP induced motor impairments were observed on the rotarod test, the platform test, and a clinical rating scale in all groups. However, each of these deficits was attenuated by AAV2-NTN (CERE-120). Stereological counts revealed a significant protection of NeuN-ir striatal neurons from 3NP toxicity by AAV2-NTN. These data support the concept that AAV2-NTN might be a valuable treatment for patients with Huntington's disease.

Authors+Show Affiliations

Department of Neuroscience, Rush University Medical Center, 1735 West Harrison Street, Suite 300, Chicago, IL 60612, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

17336076

Citation

Ramaswamy, Shilpa, et al. "Neurturin Gene Therapy Improves Motor Function and Prevents Death of Striatal Neurons in a 3-nitropropionic Acid Rat Model of Huntington's Disease." Neurobiology of Disease, vol. 26, no. 2, 2007, pp. 375-84.
Ramaswamy S, McBride JL, Herzog CD, et al. Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease. Neurobiol Dis. 2007;26(2):375-84.
Ramaswamy, S., McBride, J. L., Herzog, C. D., Brandon, E., Gasmi, M., Bartus, R. T., & Kordower, J. H. (2007). Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease. Neurobiology of Disease, 26(2), 375-84.
Ramaswamy S, et al. Neurturin Gene Therapy Improves Motor Function and Prevents Death of Striatal Neurons in a 3-nitropropionic Acid Rat Model of Huntington's Disease. Neurobiol Dis. 2007;26(2):375-84. PubMed PMID: 17336076.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease. AU - Ramaswamy,Shilpa, AU - McBride,Jodi L, AU - Herzog,Christopher D, AU - Brandon,Eugene, AU - Gasmi,Mehdi, AU - Bartus,Raymond T, AU - Kordower,Jeffrey H, Y1 - 2007/01/25/ PY - 2006/10/10/received PY - 2007/01/10/revised PY - 2007/01/14/accepted PY - 2007/3/6/pubmed PY - 2007/6/29/medline PY - 2007/3/6/entrez SP - 375 EP - 84 JF - Neurobiology of disease JO - Neurobiol Dis VL - 26 IS - 2 N2 - Huntington's disease (HD) is a devastating neurodegenerative disease characterized by the selective loss of neurons in the striatum and cerebral cortex. This study tested the hypothesis that an adenoassociated viral (AAV2) vector encoding for the trophic factor neurturin (NTN) could provide neuroprotection in the rat 3-nitropropionic acid (3NP) model of HD. Rats received AAV2-NTN (CERE-120), AAV2-eGFP or Vehicle, followed 4 weeks later by the mitochondrial toxin 3NP. 3NP induced motor impairments were observed on the rotarod test, the platform test, and a clinical rating scale in all groups. However, each of these deficits was attenuated by AAV2-NTN (CERE-120). Stereological counts revealed a significant protection of NeuN-ir striatal neurons from 3NP toxicity by AAV2-NTN. These data support the concept that AAV2-NTN might be a valuable treatment for patients with Huntington's disease. SN - 0969-9961 UR - https://www.unboundmedicine.com/medline/citation/17336076/Neurturin_gene_therapy_improves_motor_function_and_prevents_death_of_striatal_neurons_in_a_3_nitropropionic_acid_rat_model_of_Huntington's_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0969-9961(07)00021-6 DB - PRIME DP - Unbound Medicine ER -