In double-transgenic mouse models non-expanded ataxin-3 not suppresses polyglutamine neurodegeneration in the SCA3 disease – in vivo evidence

2009 ◽  
Vol 36 (S 02) ◽  
Author(s):  
J Hübener ◽  
O Riess
PLoS ONE ◽  
2013 ◽  
Vol 8 (4) ◽  
pp. e62132 ◽  
Author(s):  
Manuel Peter ◽  
Brice Bathellier ◽  
Bruno Fontinha ◽  
Pinelopi Pliota ◽  
Wulf Haubensak ◽  
...  

2000 ◽  
Vol 278 (2) ◽  
pp. H652-H657 ◽  
Author(s):  
Frank Wiesmann ◽  
Jan Ruff ◽  
Karl-Heinz Hiller ◽  
Eberhard Rommel ◽  
Axel Haase ◽  
...  

Cardiovascular transgenic mouse models with an early phenotype or even premature death require noninvasive imaging methods that allow for accurate visualization of cardiac morphology and function. Thus the purpose of our study was to assess the feasibility of magnetic resonance imaging (MRI) to characterize cardiac function and mass in newborn, juvenile, and adult mice. Forty-five C57bl/6 mice from seven age groups (3 days to 4 mo after birth) were studied by MRI under isoflurane anesthesia. Electrocardiogram-gated cine MRI was performed with an in-plane resolution of (78–117 μm)2. Temporal resolution per cine frame was 8.6 ms. MRI revealed cardiac anatomy in mice from all age groups with high temporal and spatial resolution. There was close correlation between MRI- and autopsy-determined left ventricular (LV) mass ( r = 0.95, SE of estimate = 9.5 mg). The increase of LV mass (range 9.6–101.3 mg), cardiac output (range 1.1–14.3 ml/min), and stroke volume (range 3.2–40.2 μl) with age could be quantified by MRI measurements. Ejection fraction and cardiac index did not change with aging. However, LV mass index decreased with increasing age ( P < 0.01). High-resolution MRI allows for accurate in vivo assessment of cardiac function in neonatal, juvenile, and adult mice. This method should be useful when applied in transgenic mouse models.


PLoS ONE ◽  
2013 ◽  
Vol 8 (3) ◽  
pp. e59869 ◽  
Author(s):  
Hye-Lim Ju ◽  
Sang Hoon Ahn ◽  
Do Young Kim ◽  
Sinhwa Baek ◽  
Sook In Chung ◽  
...  

2005 ◽  
Vol 175 (1) ◽  
pp. 319-328 ◽  
Author(s):  
Amanda L. Gavin ◽  
Bao Duong ◽  
Patrick Skog ◽  
Djemel Aït-Azzouzene ◽  
David R. Greaves ◽  
...  

2000 ◽  
Vol 57 (4) ◽  
pp. 1299-1306 ◽  
Author(s):  
Damien Le Menuet ◽  
Maria-Christina Zennaro ◽  
Say Viengchareun ◽  
Marc Lombès

2021 ◽  
pp. 107385842110017
Author(s):  
Laura Trujillo-Estrada ◽  
Elisabeth Sanchez-Mejias ◽  
Raquel Sanchez-Varo ◽  
Juan Antonio Garcia-Leon ◽  
Cristina Nuñez-Diaz ◽  
...  

Alzheimer’s disease (AD) is an incurable neurodegenerative disease affecting over 45 million people worldwide. Transgenic mouse models have made remarkable contributions toward clarifying the pathophysiological mechanisms behind the clinical manifestations of AD. However, the limited ability of these in vivo models to accurately replicate the biology of the human disease have precluded the translation of promising preclinical therapies to the clinic. In this review, we highlight several major pathogenic mechanisms of AD that were discovered using transgenic mouse models. Moreover, we discuss the shortcomings of current animal models and the need to develop reliable models for the sporadic form of the disease, which accounts for the majority of AD cases, as well as human cellular models to improve success in translating results into human treatments.


1997 ◽  
Vol 98 (3) ◽  
pp. 189-202 ◽  
Author(s):  
Jan Vijg ◽  
Martijn E.T Dollé ◽  
Hans-Jörg Martus ◽  
Michael E.T.I Boerrigter

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