Current Concepts of Neurodegenerative Diseases

Neurodegenerative diseases (NDDs) are described as disorders with selective loss of neurons and distinct involvement of functional systems defining clinical presentation. Extensive studies demonstrated that proteins with altered physicochemical properties are deposited in the human brain in NDDs. Th...

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Main Author: Gabor G. Kovacs
Format: Article
Language:English
Published: European Medical Journal 2013-07-01
Series:European Medical Journal Neurology
Subjects:
Online Access:http://emjreviews.com/wp-content/uploads/Current-Concepts-Of-Neurodegenerative-Diseases.pdf
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author Gabor G. Kovacs
author_facet Gabor G. Kovacs
author_sort Gabor G. Kovacs
collection DOAJ
description Neurodegenerative diseases (NDDs) are described as disorders with selective loss of neurons and distinct involvement of functional systems defining clinical presentation. Extensive studies demonstrated that proteins with altered physicochemical properties are deposited in the human brain in NDDs. The present review focuses on predominantly sporadic disorders in adulthood. Major concepts of NDDs can be summarised as follows: 1) molecular pathologic classification of NDDs is protein-based; 2) the proteinopathy concept underpins the role of protein processing systems; 3) seeding of pathological proteins supports the theory of prion-like spreading; 4) the clinical symptoms are determined by the system affected and do not unequivocally reflect the molecular pathological background; 5) overlapping of NDDs may be more the rule than the exception. Accordingly, NDD-related proteins and their biochemical modifications can be used as biomarkers and may be targeted for therapy. However, due to the high number of combinations of different proteinopathies a personalised therapy strategy may be warranted.
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spelling doaj.art-e740003205044cb4be19d6146c8abaf12022-12-21T20:48:09ZengEuropean Medical JournalEuropean Medical Journal Neurology2054-45292013-07-01217886Current Concepts of Neurodegenerative DiseasesGabor G. Kovacs0Institute of Neurology, Medical University of Vienna, Vienna, AustriaNeurodegenerative diseases (NDDs) are described as disorders with selective loss of neurons and distinct involvement of functional systems defining clinical presentation. Extensive studies demonstrated that proteins with altered physicochemical properties are deposited in the human brain in NDDs. The present review focuses on predominantly sporadic disorders in adulthood. Major concepts of NDDs can be summarised as follows: 1) molecular pathologic classification of NDDs is protein-based; 2) the proteinopathy concept underpins the role of protein processing systems; 3) seeding of pathological proteins supports the theory of prion-like spreading; 4) the clinical symptoms are determined by the system affected and do not unequivocally reflect the molecular pathological background; 5) overlapping of NDDs may be more the rule than the exception. Accordingly, NDD-related proteins and their biochemical modifications can be used as biomarkers and may be targeted for therapy. However, due to the high number of combinations of different proteinopathies a personalised therapy strategy may be warranted.http://emjreviews.com/wp-content/uploads/Current-Concepts-Of-Neurodegenerative-Diseases.pdfBiomarkerconcomitant pathologyneurodegenerative diseaseprion-likeproteinopathy
spellingShingle Gabor G. Kovacs
Current Concepts of Neurodegenerative Diseases
European Medical Journal Neurology
Biomarker
concomitant pathology
neurodegenerative disease
prion-like
proteinopathy
title Current Concepts of Neurodegenerative Diseases
title_full Current Concepts of Neurodegenerative Diseases
title_fullStr Current Concepts of Neurodegenerative Diseases
title_full_unstemmed Current Concepts of Neurodegenerative Diseases
title_short Current Concepts of Neurodegenerative Diseases
title_sort current concepts of neurodegenerative diseases
topic Biomarker
concomitant pathology
neurodegenerative disease
prion-like
proteinopathy
url http://emjreviews.com/wp-content/uploads/Current-Concepts-Of-Neurodegenerative-Diseases.pdf
work_keys_str_mv AT gaborgkovacs currentconceptsofneurodegenerativediseases