scholarly journals Long-Term Rescue of Retinal Structure and Function by Rhodopsin RNA Replacement with a Single Adeno-Associated Viral Vector in P23H RHO Transgenic Mice

2012 ◽  
Vol 23 (4) ◽  
pp. 356-366 ◽  
Author(s):  
Haoyu Mao ◽  
Marina S. Gorbatyuk ◽  
Brian Rossmiller ◽  
William W. Hauswirth ◽  
Alfred S. Lewin
Keyword(s):  
Transgenic Mice ◽  
Viral Vector ◽  
Structure And Function ◽  
Retinal Structure ◽  
And Function

scholarly journals Altered retinal structure and function in Spinocerebellar ataxia type 3

2022 ◽  
Author(s):  
Vasileios Toulis ◽  
Ricardo Casaroli-Marano ◽  
Anna Camos-Carreras ◽  
Marc Figueras-Roca ◽  
Bernardo Sanchez-Dalmau ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Spinocerebellar Ataxia ◽  
Neurodegenerative Disorder ◽  
Retinal Pigment ◽  
Structure And Function ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 3 ◽  
Retinal Structure ◽  
And Function ◽  
Type 3

Spinocerebellar ataxia type 3 is an autosomal dominant neurodegenerative disorder caused by expansion of a polyglutamine (polyQ)-encoding CAG repeat in the ATXN3 gene. Because the ATXN3 protein regulates photoreceptor ciliogenesis and phagocytosis, we aimed to explore whether expanded polyQ ATXN3 impacts retinal function and integrity in SCA3 patients and transgenic mice. We evaluated the retinal structure and function in five patients with Spinocerebellar ataxia type 3 and in a transgenic mouse model of this disease (YACMJD84.2, Q84) using, respectively, optical coherence tomography (OCT) and electroretinogram (ERG). We further determined in the transgenic mice: a) the retinal expression pattern of ATXN3 and assessed the distribution of cones and rods by immunofluorescence (IF); and b) the retinal ultrastructure by transmission electron microscopy (TEM). Some patients with Spinocerebellar ataxia type 3 in our cohort revealed: i) reduced central macular thickness indirectly correlated with disease duration; ii) decreased thickness of the macula and the ganglion cell layer, and reduced macula volume inversely correlated with disease severity (SARA score); and iii) electrophysiological dysfunction of cones, rods, and inner retinal cells. Transgenic mice replicated the human OCT and ERG findings with aged homozygous Q84/Q84 mice showing a stronger phenotype accompanied by further thinning of the outer nuclear layer and photoreceptor layer and highly reduced cone and rod activities, thus supporting severe retinal dysfunction in these mice. In addition, Q84 mice showed progressive accumulation of ATXN3-positive aggregates throughout several retinal layers and depletion of cones alongside the disease course. TEM analysis of aged Q84/Q84 mouse retinas supported the IF ATXN3 aggregation findings by revealing the presence of high number of negative electron dense puncta in ganglion cells, inner plexiform and inner nuclear layers, and further showed thinning of the outer plexiform layer, thickening of the retinal pigment epithelium and elongation of apical microvilli. Our results indicate that retinal alterations detected by non-invasive eye examination using OCT and ERG could represent a biological marker of disease progression and severity in patients with Spinocerebellar ataxia type 3.

Lung Structure And Function Relation In Elastase-Treated Rats: A Long-Term Follow Up Study

2012 ◽  
Author(s):  
Margit V. Szabari ◽  
Jozsef Tolnai ◽  
Balazs Maar ◽  
Harikrishnan Parameswaran ◽  
Elizabeth Bartolak-Suki ◽  
...  
Keyword(s):  
Structure And Function ◽  
Follow Up Study ◽  
Lung Structure ◽  
Long Term Follow Up ◽  
And Function ◽  
Function Relation

Abstract P138: Long-term Effects Of Severe Caloric Restriction To The Heart Function

Hypertension ◽  
2021 ◽  
Vol 78 (Suppl_1) ◽  
Author(s):  
Aline M De Souza ◽  
Jonathas Almeida ◽  
Nataliia Shults ◽  
Hong Ji ◽  
Kathryn Sandberg
Keyword(s):  
Cardiovascular Disease ◽  
Caloric Restriction ◽  
Heart Function ◽  
Left Ventricular ◽  
Structure And Function ◽  
Long Term Effects ◽  
Isolated Hearts ◽  
Ad Libitum ◽  
And Function

Severe caloric restriction (sCR) increases the risk for acute cardiovascular disease. Less understood are the long-term effects on cardiovascular disease risk after the sCR period has ended. We investigated the effects of sCR on heart structure and function months after refeeding (sCR-Refed). Female Fischer rats (3-months-old) were maintained on (CT) ad libitum or a 60% caloric restricted diet for 2 weeks. Thereafter, all rats received ad libitum chow for 3 months and they were analyzed by precision ultrasound to assess their heart function. After imaging, the animals were sacrificed and the hearts were subjected to ischemia-reperfusion (I/R) using a Langendorff preparation. After 2 weeks of sCR, rats lost 15% of their initial body weight (BW) [% (100*(Final-Initial/Initial)): CT, 1.5±0.8 vs sCR, -15.4±1.1; p<0.001;n=8]. After 3 months of refeeding, there was no detectable difference in BW between CT and sFR-Refed groups. Isolated hearts from the sCR-Refed rats exhibited worse myocardial pathology after I/R compared to CT rats. The parallel orientation of myofibers and striations normally present in cardiomyocytes was lost in sCR-Refed rats. Further analysis revealed uneven blood-filling of the microcirculatory vessels and prominent interstitial edema of the myocardium. Hearts from sCR-Refed rats had more atrophied cardiomyocytes than CT [Atrophied/Total (%): CT, 0.2±0.1 vs sCR-Refed, 50.6±1.1; p<0.001; n=5]. The number of arrhythmic events during a 30 min ischemic interval in isolated hearts doubled after 2 weeks on the sCR diet ( data not shown ) and remained doubled 3 months later [Arrhythmias (% of time): CT, 34±8 vs sCR-Refed, 68±9; p=0.02; n=8]. Ultrasound imaging showed no difference in stroke volume, coronary perfusion pressure and left ventricular mass. However, the thickness of the left ventricular posterior wall was significantly reduced in sCR-Refed rats [(mm): CT, 2.55 ±0.03 vs sCR-Refed, 2.10±0.04; p=0.002; n=4]. These findings indicate heart structure and function remained damaged months after the sCR period ended and BW was restored. These studies have adverse cardiovascular risk implications for who are subjected either voluntarily (crash diets) or involuntarily (very low food security) to periods of inadequate caloric intake.

Long-term changes in structure and function of a tropical headwater stream following a disease-driven amphibian decline

2014 ◽  
Vol 60 (3) ◽  
pp. 575-589 ◽  
Author(s):  
Heidi M. Rantala ◽  
Amanda M. Nelson ◽  
Jessica N. Fulgoni ◽  
Matt R. Whiles ◽  
Robert O. Hall ◽  
...  
Keyword(s):  
Amphibian Decline ◽  
Structure And Function ◽  
Headwater Stream ◽  
Long Term Changes ◽  
And Function

Retinal Structure and Function in Perinatally HIV-Infected and cART-Treated Children: A Matched Case–Control Study

2015 ◽  
Vol 56 (6) ◽  
pp. 3945 ◽  
Author(s):  
Nazli Demirkaya ◽  
Sophie Cohen ◽  
Ferdinand W. N. M. Wit ◽  
Michael D. Abramoff ◽  
Reinier O. Schlingemann ◽  
...  
Keyword(s):  
Case Control Study ◽  
Case Control ◽  
Structure And Function ◽  
Matched Case ◽  
Retinal Structure ◽  
And Function ◽  
Matched Case Control Study ◽  
Control Study

scholarly journals Alterations of the thyroid structure and function in experimental hypopinealism

2011 ◽  
Vol 57 (2) ◽  
pp. 32-35
Author(s):  
L A Bondarenko ◽  
L Iu Sergienko ◽  
N N Sotnik ◽  
A N Cherevko
Keyword(s):  
Thyroid Gland ◽  
Structural Changes ◽  
Structure And Function ◽  
Initial Increase ◽  
Histological Structure ◽  
Pituitary Thyroid Axis ◽  
Thyroid Axis ◽  
And Function ◽  
Sexually Mature

The pituitary-thyroid axis of young sexually mature rabbits kept under a 24-hour daylight photoperiod was shown to undergo phase-modulated variations of hormonal activity with its initial increase (during the first month) and subsequent progressive decrease (within 2-5 months after the onset of exposure to light). These changes correlated with the time-dependent fall in the blood T3, T4, and TSH levels. Simultaneously, the animals developed pathological changes in the histological structure of the thyroid gland similar to those in patients with secondary or tertiary hypothyroidism. It is concluded that hormonal and structural changes in the thyroid gland during long-term hypopinealism should be regarded as an experimental model of hypothyroidism of neuroendocrine origin.

scholarly journals Retinal Structure and Function in Achromatopsia

Ophthalmology ◽  
2014 ◽  
Vol 121 (1) ◽  
pp. 234-245 ◽  
Author(s):  
Venki Sundaram ◽  
Caroline Wilde ◽  
Jonathan Aboshiha ◽  
Jill Cowing ◽  
Colin Han ◽  
...  
Keyword(s):  
Structure And Function ◽  
Retinal Structure ◽  
And Function
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