The Role of Chaperones and Co-Chaperones in Retinal Degenerative Diseases

2008 ◽  
pp. 109-123
Author(s):  
Maria Kosmaoglou ◽  
Tatiana V. Novoselova ◽  
Michael E. Cheetham
Keyword(s):  
Degenerative Diseases ◽  
Retinal Degenerative Diseases

scholarly journals Transplanted embryonic retinal stem cells have the potential to repair the injured retina in mice

2021 ◽  
Author(s):  
Xia Feng ◽  
Peng Chen ◽  
Xin Zhao ◽  
Jing Wang ◽  
Hong Wang
Keyword(s):  
Stem Cells ◽  
Optic Nerve ◽  
Intravitreal Injection ◽  
Brdu Incorporation ◽  
Positive Staining ◽  
Degenerative Diseases ◽  
Nerve Crush ◽  
Body Tissues ◽  
Retinal Degenerative Diseases

Abstract Background: Stem cell transplantation has been reported as one of the promising strategies to treat retinal degenerative diseases. But, the application and the role of retina stem cells (RSCs) in the treatment of patients with retinal degenerative diseases have not been fully revealed. This study aimed to investigate the potential role of transplantation of the embryo-derived RSCs into the vitreous cavity in repairing the damaged retina in mice.Methods: RSCs were isolated from Kunming mice E17 embryonic retina and ciliary body tissues, and labeled with 5-bromo-2’-deoxyuridin (BrdU). Retinal optic nerve crush injury was induced in left eyes in male Kunming mice by ring clamping the optic nerve. The 6th-generation of BrdU-labeled RSCs were transplanted into the damaged retina by the intravitreal injection, and saline injected eyes were used as the control. Hematoxylin and eosin histological staining, and BrdU, Nestin and Pax6 immunostaining were performed. Electroretinogram (ERG) was used for assessing the electrical activity of the retina.Results: Embryo-derived RSCs were identified by the positive stains of Pax6 and Nestin. BrdU incorporation was detected in the majority of RSCs. The damaged retina showed cellular nuclear disintegration and fragmentation in the retinal tissue which progressed over the periods of clamping time, and decreased amplitudes of a and b waves in ERG. In the damaged retina with RSCs transplantation, the positive staining for BrdU, Pax6 and Nestin were revealed on the retinal surface. Notably, RSCs migrated into the retinal ganglion cell layer and inner nuclear. Transplanted RSCs significantly elevated the amplitudes of a waves in retina injured eyes.Conclusions: Embryonic RSCs have similar characteristics to neural stem cells. Transplantation of RSCs by intravitreal injection would be able to repair the damaged retina.

scholarly journals Transplanted embryonic retinal stem cells have the potential to repair the injured retina in mice

2020 ◽  
Author(s):  
Xia Feng ◽  
Peng Chen ◽  
Xin Zhao ◽  
Jing Wang ◽  
Hong Wang
Keyword(s):  
Stem Cells ◽  
Intravitreal Injection ◽  
Brdu Incorporation ◽  
Positive Staining ◽  
Degenerative Diseases ◽  
Retinal Injury ◽  
Nuclear Disintegration ◽  
Body Tissues ◽  
Retinal Degenerative Diseases

Abstract Background: Stem cell transplantation has been reported as one of the promising strategies to treat retinal degenerative diseases. But, the application and the role of retina stem cells (RSCs) in the treatment of patients with retinal degenerative diseases have not been fully revealed. This study aimed to investigate the potential role of transplantation of the embryo-derived RSCs into the vitreous cavity in repairing the damaged retina in mice. Methods: RSCs were isolated from Kunming mice E17 embryonic retina and ciliary body tissues, and labeled with 5-bromo-2’-deoxyuridin (BrdU). Retinal injury was induced in left eyes in male Kunming mice by ring clamping the optic nerve. The 6th-generation of BrdU-labeled RSCs were transplanted into the damaged retina by the intravitreal injection, and saline injected eyes were used as the control. Hematoxylin and eosin histological staining, and BrdU, Nestin and Pax6 immunostaining were performed. Electroretinogram (ERG) was used for assessing the electrical activity of the retina. Results: Embryo-derived RSCs were identified by the positive stains of Pax6 and Nestin. BrdU incorporation was detected in the majority of RSCs. The damaged retina showed cellular nuclear disintegration and fragmentation in the retinal tissue which progressed over the periods of clamping time, and decreased amplitudes of a and b waves in ERG. In the damaged retina with RSCs transplantation, the positive staining for BrdU, Pax6 and Nestin were revealed on the retinal surface. Notably, a small amount of RSCs migrated into the retinal ganglion cell layer and inner nuclear. Transplanted RSCs significantly elevated the amplitudes of a waves in retina injured eyes. Conclusions: Embryonic RSCs have similar characteristics to neural stem cells. Transplantation of RSCs by intravitreal injection would be able to repair the damaged retina.

scholarly journals The Role of Inflammation in Retinal Neurodegeneration and Degenerative Diseases

2021 ◽  
Vol 23 (1) ◽  
pp. 386
Author(s):  
Geetika Kaur ◽  
Nikhlesh K. Singh
Keyword(s):  
Retinal Degeneration ◽  
Vision Loss ◽  
Age Related Macular Degeneration ◽  
Treatment Modalities ◽  
Macular Dystrophy ◽  
Degenerative Diseases ◽  
Age Related ◽  
Retinal Neurodegeneration ◽  
Retinal Degenerative Diseases

Retinal neurodegeneration is predominantly reported as the apoptosis or impaired function of the photoreceptors. Retinal degeneration is a major causative factor of irreversible vision loss leading to blindness. In recent years, retinal degenerative diseases have been investigated and many genes and genetic defects have been elucidated by many of the causative factors. An enormous amount of research has been performed to determine the pathogenesis of retinal degenerative conditions and to formulate the treatment modalities that are the critical requirements in this current scenario. Encouraging results have been obtained using gene therapy. We provide a narrative review of the various studies performed to date on the role of inflammation in human retinal degenerative diseases such as age-related macular degeneration, inherited retinal dystrophies, retinitis pigmentosa, Stargardt macular dystrophy, and Leber congenital amaurosis. In addition, we have highlighted the pivotal role of various inflammatory mechanisms in the progress of retinal degeneration. This review also offers an assessment of various therapeutic approaches, including gene-therapies and stem-cell-based therapies, for degenerative retinal diseases.

scholarly journals Role of Microglia Adenosine A2AReceptors in Retinal and Brain Neurodegenerative Diseases

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Ana R. Santiago ◽  
Filipa I. Baptista ◽  
Paulo F. Santos ◽  
Gonçalo Cristóvão ◽  
António F. Ambrósio ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Therapeutic Strategy ◽  
Therapeutic Potential ◽  
Brain Diseases ◽  
Degenerative Diseases ◽  
Chronic Neuroinflammation ◽  
Retinal Degenerative Diseases ◽  
G Protein Coupled ◽  
The Brain

Neuroinflammation mediated by microglial cells in the brain has been commonly associated with neurodegenerative diseases. Whether this microglia-mediated neuroinflammation is cause or consequence of neurodegeneration is still a matter of controversy. However, it is unequivocal that chronic neuroinflammation plays a role in disease progression and halting that process represents a potential therapeutic strategy. The neuromodulator adenosine emerges as a promising targeting candidate based on its ability to regulate microglial proliferation, chemotaxis, and reactivity through the activation of its G protein coupledA2Areceptor (A2AR). This is in striking agreement with the ability ofA2ARblockade to control several brain diseases. Retinal degenerative diseases have been also associated with microglia-mediated neuroinflammation, but the role ofA2ARhas been scarcely explored. This review aims to compare inflammatory features of Parkinson’s and Alzheimer’s diseases with glaucoma and diabetic retinopathy, discussing the therapeutic potential ofA2ARin these degenerative conditions.

scholarly journals A Novel HIF Inhibitor Halofuginone Prevents Neurodegeneration in a Murine Model of Retinal Ischemia-Reperfusion

2019 ◽  
Vol 20 (13) ◽  
pp. 3171 ◽  
Author(s):  
Hiromitsu Kunimi ◽  
Yukihiro Miwa ◽  
Hiroyoshi Inoue ◽  
Kazuo Tsubota ◽  
Toshihide Kurihara
Keyword(s):  
Intraocular Pressure ◽  
Neuroprotective Effect ◽  
Ischemia Reperfusion ◽  
Hypoxia Inducible Factor ◽  
Retinal Ischemia ◽  
In Vitro Screening ◽  
Degenerative Diseases ◽  
Retinal Degenerative Diseases

Neurodegeneration caused with retinal ischemia or high intraocular pressure is irreversible in general. We have focused on the role of hypoxia-inducible factor (HIF) in retinal homeostasis and revealed that HIF inhibition may be effective against retinal neovascular and neurodegeneration. In this study, we performed in vitro screening of natural products and found halofuginone, which is a derivative of febrifugine extracted from hydrangea, as a novel HIF inhibitor. Administration of halofuginone showed a significant neuroprotective effect by inhibiting HIF-1α expression in a murine retinal ischemia-reperfusion model histologically and functionally. These results indicate that halofuginone can be a neuroprotective agent in ischemic retinal degenerative diseases.

scholarly journals Transplanted embryonic retinal stem cells have the potential to repair the injured retina in mice

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xia Feng ◽  
Peng Chen ◽  
Xin Zhao ◽  
Jing Wang ◽  
Hong Wang
Keyword(s):  
Stem Cells ◽  
Optic Nerve ◽  
Intravitreal Injection ◽  
Brdu Incorporation ◽  
Positive Staining ◽  
Degenerative Diseases ◽  
Nerve Crush ◽  
Body Tissues ◽  
Retinal Degenerative Diseases

Abstract Background Stem cell transplantation has been reported as one of the promising strategies to treat retinal degenerative diseases. But, the application and the role of retina stem cells (RSCs) in the treatment of patients with retinal degenerative diseases have not been fully revealed. This study aimed to investigate the potential role of transplantation of the embryo-derived RSCs into the vitreous cavity in repairing the damaged retina in mice. Methods RSCs were isolated from Kunming mice E17 embryonic retina and ciliary body tissues, and labeled with 5-bromo-2’-deoxyuridin (BrdU). Retinal optic nerve crush injury was induced in left eyes in male Kunming mice by ring clamping the optic nerve. The 6th -generation of BrdU-labeled RSCs were transplanted into the damaged retina by the intravitreal injection, and saline injected eyes were used as the control. Hematoxylin and eosin histological staining, and BrdU, Nestin and Pax6 immunostaining were performed. Electroretinogram (ERG) was used for assessing the electrical activity of the retina. Results Embryo-derived RSCs were identified by the positive stains of Pax6 and Nestin. BrdU incorporation was detected in the majority of RSCs. The damaged retina showed cellular nuclear disintegration and fragmentation in the retinal tissue which progressed over the periods of clamping time, and decreased amplitudes of a and b waves in ERG. In the damaged retina with RSCs transplantation, the positive staining for BrdU, Pax6 and Nestin were revealed on the retinal surface. Notably, RSCs migrated into the retinal ganglion cell layer and inner nuclear. Transplanted RSCs significantly elevated the amplitudes of a waves in retina injured eyes. Conclusions Embryonic RSCs have similar characteristics to neural stem cells. Transplantation of RSCs by intravitreal injection would be able to repair the damaged retina.

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