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Life
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New Insights on Cellular Biology of Retinal Degenerations
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New Insights on Cellular Biology of Retinal Degenerations

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Physiology and Pathology".

Deadline for manuscript submissions: closed (26 August 2022) | Viewed by 14290

Special Issue Editors

Dr. Luigi Donato

E-Mail Website
Guest Editor
Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, 98125 Messina, Italy
Interests: oxidative stress; inherited retinal dystrophies (IRDs); retinitis pigmentosa (RP); cerebral cavernous malformations (CCMs); trimethylaminuria (TMAU)
Special Issues, Collections and Topics in MDPI journals
Dr. Concetta Scimone

E-Mail Website
Guest Editor
Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, 98125 Messina, Italy
Interests: angiogenesis; neurovascular unit; blood-brain barrier; cerebrovascular disease; small vessel malformations; inherited retinal dystrophies; oxidative stress
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Misfolded host protein accumulation is crucial to the neuropathogenesis of numerous human brain diseases. Among them, inherited retinal degenerations (IRDs) represent one of the most recent groups of neural diseases associated with cellular protein homeostasis (proteostasis) disruption. Today, several cellular processes are known to be at the base of this impairment: inflammation, mitochondrion alterations, RNA processing, splicing, circadian rhythms, epigenetic modifications, fatty acid metabolism, oxidative stress, apoptosis and cell death, cytoskeleton rearrangements and vesicular trafficking, and signal transduction phototransduction. The interaction between different chaperones can be altered by oxidative stress, one of the most well-described causes of retinal cell-induced death, due to activation/deactivation switches that induce certain chaperones—encoding genes and repressing others. Endoplasmic reticulum (ER) stress and unfolded protein response (UPR) signaling have been implicated in the etiopathogenesis of heritable forms of retinitis pigmentosa. UPR determines an initial inhibition of translation to prevent further accumulation of misfolded proteins, and an upregulation of chaperone genes to improve protein folding, followed by activation of the ER-associated degradation system, which retro-translocates misfolded proteins from the ER for proteasome-dependent degradation. If ER stress persists, UPR signaling “switches” to trigger cell death by activating the intrinsic apoptosis pathway. Photoreceptors maintain specific structure and dimensions via continuous renewal of outer segments, while simultaneously old and outer waste segments, rich in misfolded proteins, are phagocytized by the retinal pigment epithelium (RPE). Nevertheless, the knowledge regarding the role of misfolded proteins into retinal survival and vision process is still limited, and there is an active field of research aiming to further identify new proteins and new pathways.

This Special Issue aims to involve researchers working on retinal cell biology, including protein misfolding and network alterations, with a focus on retinal degenerations. Contributors may submit research articles, reviews, or original papers describing the identification of new misfolded proteins, their accumulation in retinal cells, their involvement in ER stress and UPR, the functional relationship with RPE, the construction of protein interaction networks, and the assembly of protein complexes formed by mutant proteins associated with retinal genetic diseases.

Dr. Luigi Donato
Dr. Concetta Scimone
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Life is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • oxidative stress
  • antioxidants
  • inherited retinal dystrophies (IRDs)
  • retinal pigment epithelium (RPE)
  • photoreceptors
  • cellular death
  • apoptosis
  • autophagy
  • cellular metabolism
  • cell cycle
  • vesicular trafficking
  • unfolded protein response (UPR)
  • endoplasmic reticulum stress
  • chaperone activity
  • small GTPase signaling
  • retinoic acid cycle
  • microvascular integrity
  • circadian rhythms
  • fatty acids metabolism
  • synapse integrity
  • retinal cells rescue
  • editome
  • cellular biomarkers
  • RNAome

Published Papers (3 papers)

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Review

16 pages, 332 KiB  
Review
Treatment of Inherited Retinal Dystrophies with Somatic Cell Therapy Medicinal Product: A Review
by Giacomo Maria Bacci, Valentina Becherucci, Elisa Marziali, Andrea Sodi, Franco Bambi and Roberto Caputo
Life 2022, 12(5), 708; https://doi.org/10.3390/life12050708 - 9 May 2022
Cited by 3 | Viewed by 1890
Abstract
Inherited retinal dystrophies and retinal degenerations related to more common diseases (i.e., age-related macular dystrophy) are a major issue and one of the main causes of low vision in pediatric and elderly age groups. Advancement and understanding in molecular biology and the possibilities [...] Read more.
Inherited retinal dystrophies and retinal degenerations related to more common diseases (i.e., age-related macular dystrophy) are a major issue and one of the main causes of low vision in pediatric and elderly age groups. Advancement and understanding in molecular biology and the possibilities raised by gene-editing techniques opened a new era for clinicians and patients due to feasible possibilities of treating disabling diseases and the reduction in their complications burden. The scope of this review is to focus on the state-of-the-art in somatic cell therapy medicinal products as the basis of new insights and possibilities to use this approach to treat rare eye diseases. Full article
(This article belongs to the Special Issue New Insights on Cellular Biology of Retinal Degenerations)
33 pages, 1599 KiB  
Review
Retinal Glutamate Neurotransmission: From Physiology to Pathophysiological Mechanisms of Retinal Ganglion Cell Degeneration
by Isabella Boccuni and Richard Fairless
Life 2022, 12(5), 638; https://doi.org/10.3390/life12050638 - 25 Apr 2022
Cited by 22 | Viewed by 6696
Abstract
Glutamate neurotransmission and metabolism are finely modulated by the retinal network, where the efficient processing of visual information is shaped by the differential distribution and composition of glutamate receptors and transporters. However, disturbances in glutamate homeostasis can result in glutamate excitotoxicity, a major [...] Read more.
Glutamate neurotransmission and metabolism are finely modulated by the retinal network, where the efficient processing of visual information is shaped by the differential distribution and composition of glutamate receptors and transporters. However, disturbances in glutamate homeostasis can result in glutamate excitotoxicity, a major initiating factor of common neurodegenerative diseases. Within the retina, glutamate excitotoxicity can impair visual transmission by initiating degeneration of neuronal populations, including retinal ganglion cells (RGCs). The vulnerability of RGCs is observed not just as a result of retinal diseases but has also been ascribed to other common neurodegenerative and peripheral diseases. In this review, we describe the vulnerability of RGCs to glutamate excitotoxicity and the contribution of different glutamate receptors and transporters to this. In particular, we focus on the N-methyl-d-aspartate (NMDA) receptor as the major effector of glutamate-induced mechanisms of neurodegeneration, including impairment of calcium homeostasis, changes in gene expression and signalling, and mitochondrial dysfunction, as well as the role of endoplasmic reticular stress. Due to recent developments in the search for modulators of NMDA receptor signalling, novel neuroprotective strategies may be on the horizon. Full article
(This article belongs to the Special Issue New Insights on Cellular Biology of Retinal Degenerations)
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14 pages, 1190 KiB  
Review
Retinal Progression Biomarkers of Early and Intermediate Age-Related Macular Degeneration
by Rita Flores, Ângela Carneiro, Sandra Tenreiro and Miguel C. Seabra
Life 2022, 12(1), 36; https://doi.org/10.3390/life12010036 - 27 Dec 2021
Cited by 13 | Viewed by 4630
Abstract
Early and intermediate AMD patients represent a heterogeneous population with an important but variable risk of progression to more advanced stages of the disease. The five-year progression from early and intermediate AMD to late disease is known to range from 0.4% to 53%. [...] Read more.
Early and intermediate AMD patients represent a heterogeneous population with an important but variable risk of progression to more advanced stages of the disease. The five-year progression from early and intermediate AMD to late disease is known to range from 0.4% to 53%. This wide variation explains the particular interest in searching predictive AMD biomarkers. Clinical parameters such as drusen size, presence of pigmentary abnormalities, and fellow eye status were, traditionally, the more important predictive elements. Multimodal retinal assessment (Color Fundus Photography, Optical Coherence Tomography, Optical Coherence Angiography and Fundus Autofluorescence) is providing new and accurate image biomarkers, useful in research and in daily practice. If individual progression risk could be anticipated, then management plans should be adapted accordingly, considering follow-up intervals and therapeutic interventions. Here, we reviewed the most important image progression biomarkers of early and intermediate AMD with relevant interest in clinical practice. Full article
(This article belongs to the Special Issue New Insights on Cellular Biology of Retinal Degenerations)
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