BackgroundThe zQ175 knock-in line was generated by replacing mouse endogenous Htt exon 1 with a mutant version of human HTT exon 1 followed by the expansion of the CAG repeat. This line is now being used extensively throughout the HD research community.MethodWe have performed a molecular ...
In the present study, we used small-animal PET to characterize the expression of molecular targets in the recently reported HD animal model, the zQ175 mouse model. Methods: Male heterozygous zQ175 (Htt(tm1Mfc)/190JChdi, CHDI-81003003) and wild-type (WT, C57BL/6J) animals were imaged ...
Carty N, Berson N, Tillack K, Thiede C, Scholz D, Kottig K, Sedaghat Y, Gabrysiak C, Yohrling G, von der Kammer H, Ebneth A, Mack V, Munoz- Sanjuan I, Kwak S (2015) Characterization of HTT inclusion size, location, and timing in the zQ175 mouse model of Huntington's ...
P. (2016). Optimising Golgi-Cox staining for use with perfusion-fixed brain tissue validated in the zQ175 mouse model of Huntington's disease. J. Neurosci. Methods 265, 81-88. doi: 10.1016/j.jneumeth.2015.09.033Bayram-Weston Z, Olsen E, Harrison DJ, Dunnett SB, Brooks SP. Optimising ...
ObjectiveThe aim of our study was to determine the steady state and dynamic in mitochondrial fission and fusion balance, in quadriceps muscle tissue of the zQ175 HD mouse model and human HTT gene expansion carriers.BackgroundMitochondrial fission and fusion processes are important for maintaining ...
Since the discovery of the gene mutation that causes the disease, mouse models have been developed by different strategies. Recently, a new model, the zQ175 knock-in (KI) line, was developed in an attempt to have the Htt gene in a context and causing a phenotype that more closely mimics...
We conducted a comparative immunofluorescence analysis in the striatum of WT and the heterozygous zQ175 HD mouse model and found that the expression and abundance of GFAP+ and S100B+ astrocytes increased in zQ175 mice, while GS+ astrocytes showed no alteration. We then explored whether there was...
To assess whether whole-brain changes in CBF are present and follow a pattern of progression, we investigated both resting-state brain perfusion and vascular reactivity longitudinally in the zQ175DN mouse model of HD.Using pseudo-continuous arterial spin labelling (pCASL) MRI in the zQ175DN ...
This mouse model shows molecular, cellular and circuitry alterations that worsen through age. Motor function disturbances are manifested in this model at 6 and 10 months of age. Specifically, we investigated, longitudinally, changes in co-activation patterns (CAPs) that are the transient states of ...
We previously reported that inhibition of Drp1 hyperactivation by P110, a rationally designed peptide inhibitor of Drp1-Fis1 interaction, is protective in the HD R6/2 mouse model, which expresses a fragment of mutant Huntingtin (mHtt). In this study, we expand our work to test the effect ...