Neurodegeneration Scientific Publications

Latest publication 05/01/2020

SCA7 mice recapitulate CNS- PNS and retina pathologies and show a transcriptiona

Here we describe the first SCA7 knock-in mice that combine most cardinal features of SCA7, including retinal, cerebellar, cerebral and peripheral...

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    [title] => SCA7 mice recapitulate CNS- PNS and retina pathologies and show a transcriptiona
    [paragraph] => SCA7 mice recapitulate CNS, PNS and retina pathologies and show a transcriptional signature of Purkinje cell dysfunction prevailing in SCA1 and SCA2 mice
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Authors
A NIEWIADOMSKA-CIMICKA, F Doussau, JB Perot et al

 Lab
Institut de Genetique et de Biologie Moleculaire et Cellulaire
Journal
Molecular Neurodegeneration
Abstract
Here we describe the first SCA7 knock-in mice that combine most cardinal features of SCA7, including retinal, cerebellar, cerebral and peripheral nerves pathologies, which account for progressive impairment of behavior, motor and vision functions. MRI and brain morphometry reveal atrophy of grey and white matter of specific cerebral regions, while peripheral nerves and photoreceptors show functional and morphological alterations. We show that cerebellar pathology is characterized by gene deregulation in all cerebellar cell types and alterations of SAGA-dependent epigenetic marks. Intranuclear accumulation of mutant ATXN7 and gene downregulation precede the onset of PC pacemaker dysfunction. Interestingly, PC show loss of expression of 83 PC-specific genes coding for ion channels, receptors and signaling proteins involved in pacemaker function and long-term depression, which are causal factors of many type of ataxias. Comparison of cerebellar transcriptome with other SCAs reveals a subset of 67 PC-specific genes downregulated in SCA1, SCA2 and SCA7, providing a common signature of early PC dysfunction.
BIOSEB Instruments Used
Aron Test or Four Plates Test (LE830),Grip strength test (BIO-GS3),Rotarod (BX-ROD)
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The Four Plates Test is an exclusivity from BIOSEB!
The Aron test allows a quick characterization of putative anxiolytics compounds in naïve animals. A must for studying anxiety, drug screening and phenotyping.
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An easy way to objectively quantify the muscular strength of mice and rats, and to assess the effect of drugs, toxins, muscular (i.e. myopathy) and neurodegenerative diseases on muscular degeneration. It is widely used in conjunction with the ROTAROD motor coordination test: a normally coordinated rodent will show a decreased latency to fall off the rotating rod if its muscular strength is low. The Grip Strength Test is a must for your research on activity, motor control & coordination, and is particularly well suited for studies on Parkinson's & Huntington's disease.
New features GS4 - 2023: Color display with permanent backlight screen for easier reading, reset by footswitch, Improved battery time, Larger data memory of 500 values, Animal counter, USB port (charging/data transfer)

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