Transcriptional Assessment of Striatal mRNAs as Valid Biomarkers of Disease Progression in Three Mouse Models of Huntington’s Disease

Abstract Background: Huntington’s disease (HD) is a progressive neurodegenerative disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral,...


Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington’s disease

Zeitler B, Froelich S, Marlen K, Shivak D.A, Yu Q, Li D, Pearl J.R, Miller J.C, Zhang L, Paschon D.E,...


Large-scale phenome analysis defines a behavioral signature for Huntington’s disease genotype in mice.

Alexandrov V, Brunner D, Menalled LB, Kudwa A, Watson-Johnson J, Mazzella M, Russel I, Ruiz MC, Torello J, Sabath E,...


Genetic Deletion of Transglutaminase 2 Does Not Rescue the Phenotypic Deficits Observed in R6/2 and zQ175 Mouse Models of Huntington’s Disease

Menalled LB, Kudwa A, Oakeshott S, Farrar A, Paterson N, Filippov I, Miller S, Kwan M, Olsen M, Beltran J,...


Regional vulnerability in Huntington’s disease: fMRI-guided molecular analysis in patients and a mouse model of disease

Moreno H, Lewandowski N, Bordelon Y, Brickman A, Angulo S, Khan U, Muraskin J, Griffith E, Wasserman P, Menalled L,...


Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington’s disease: zQ175

Menalled L, Kudwa A, Miller S, Fitzpatrick J, Watson-Johnson J, Keating N, Ruiz M, Mushlin R, Alosio B, McConnell K,...


Effect of the rd1 mutation on motor performance in R6/2 and wild type mice. PLoS Currents Huntington Disease.

Menalled, L., El-Khodor, B. F., Hornberger, M., Park, L., Howland, D., and Brunner, D.


Caspase-6 activity in a BAC HD mouse modulates steady state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.

Juliette Gafni, Theodora Papanikolaou, Francesco DeGiacomo, Jennifer Holcomb, Sylvia Chen, Menalled L.; Kudwa A.; Jon Fitzpatrick, Miller S.; Ramboz S.;...


HD mouse models reveal clear deficits in learning to perform a simple instrumental response. PLoS Currents: Huntington Disease.

Oakeshott, S., Port, R. G., Cummins-Sutphen, J., Watson-Johnson, J., Ramboz, S., Park, L., Howland, D., and Brunner, D.

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