The ubiquitin-proteasome system in retinal health and disease

The ubiquitin–proteasome system (UPS) is the main intracellular pathway for modulated protein turnover, playing an important role in the maintenance of cellular homeostasis. It also exerts a protein quality control through degradation of oxidized, mutant, denatured, or misfolded proteins and is involved in many biological processes where protein level regulation is necessary. This system allows the cell to modulate its protein expression pattern in response to changing physiological conditions and provides a critical protective role in health and disease. Impairments of UPS function in the central nervous system (CNS) underlie an increasing number of genetic and idiopathic diseases, many of which affect the retina. Current knowledge on the UPS composition and function in this tissue, however, is scarce and dispersed. This review focuses on UPS elements reported in the retina, including ubiquitinating and deubiquitinating enzymes (DUBs), and alternative proteasome assemblies. Known and inferred roles of protein ubiquitination, and of the related, SUMO conjugation (SUMOylation) process, in normal retinal development and adult homeostasis are addressed, including modulation of the visual cycle and response to retinal stress and injury. Additionally, the relationship between UPS dysfunction and human neurodegenerative disorders affecting the retina, including Alzheimer's, Parkinson's, and Huntington's diseases, are dealt with, together with numerous instances of retina-specific illnesses with UPS involvement, such as retinitis pigmentosa, macular degenerations, glaucoma, diabetic retinopathy (DR), and aging-related impairments. This information, though still basic and limited, constitutes a suitable framework to be expanded in incoming years and should prove orientative toward future therapy design targeting sight-affecting diseases with a UPS underlying basis.

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Mode of access: Internet.

Neurological bulletin. Clinical studies of nervous and mental diseases in the Neurological Department of Columbia University.

Editor: Frederick Tilney.

References to nerves : being tabular views of the motor nerves to muscles, the nervous supply of the principal articulations and of the skin, the deep and superficial origins of the cranial nerves; with the most important effects following solution of continuity of nerves.

Mode of access: Internet.

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Signatures: pi⁸ A-2I⁸ K⁴.

A manual of psychological medicine and allied nervous diseases : containing the description, etiology, diagnosis, pathology and treatment of insanity, with especial reference to the clinical features of mental diseases and the allied neuroses and its medico-legal aspects with a carefully prepared digest of the lunacy laws in the various states relating to the care, custody and responsibility of the insane /

Includes bibliographical references and index.

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1871 ed. has title: The physics and physiology of spiritualism; 1881 ed. has title: On certain conditions of nervous derangement.

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Mode of access: Internet.

Nervous and mental disease monograph series

Mode of access: Internet.

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Mode of access: Internet.

Text-book of nervous diseases, being a compendium for the use of students and practitioners of medicine

Mode of access: Internet.

Functional nervous diseases:

Mode of access: Internet.

The English malady, or, A treatise of nervous diseases of all kinds : as spleen, vapours, lowness of spirits, hypochondriacal, and hysterical distempers, &c., in three parts: Part I. Of the nature and cause of nervous distempers : Part II. Of the cure of nervous distempers : Part III. Variety of cases that illustrate and confirm the method of cure : with the author's own case at large /

Each part has special title page.

Manual of nervous diseases

Includes bibliographical references (p. 309-310) and index.