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Hypo- and Hyper-Assembly Diseases of RNA-Protein Complexes.
- Source :
-
Trends in molecular medicine [Trends Mol Med] 2016 Jul; Vol. 22 (7), pp. 615-628. Date of Electronic Publication: 2016 Jun 03. - Publication Year :
- 2016
-
Abstract
- A key aspect of cellular function is the proper assembly and utilization of ribonucleoproteins (RNPs). Recent studies have shown that hyper- or hypo-assembly of various RNPs can lead to human diseases. Defects in the formation of RNPs lead to 'RNP hypo-assembly diseases', which can be caused by RNA degradation outcompeting RNP assembly. By contrast, excess RNP assembly, either in higher order RNP granules, or due to the expression of repeat-containing RNAs, can lead to 'RNP hyper-assembly diseases'. Here, we discuss the most recent advances in understanding the cause of disease onset, as well as potential therapies from the aspect of modulating RNP assembly in the cell, which presents a novel route to the treatment of these diseases.<br /> (Copyright © 2016 Elsevier Ltd. All rights reserved.)
- Subjects :
- Animals
Dwarfism genetics
Dwarfism metabolism
Dwarfism pathology
Dyskeratosis Congenita genetics
Dyskeratosis Congenita pathology
Fetal Growth Retardation genetics
Fetal Growth Retardation metabolism
Fetal Growth Retardation pathology
Hair abnormalities
Hair metabolism
Hair pathology
Hirschsprung Disease genetics
Hirschsprung Disease metabolism
Hirschsprung Disease pathology
Humans
Immunologic Deficiency Syndromes genetics
Immunologic Deficiency Syndromes metabolism
Immunologic Deficiency Syndromes pathology
Mallory Bodies genetics
Mallory Bodies metabolism
Mallory Bodies pathology
Microcephaly genetics
Microcephaly metabolism
Microcephaly pathology
Muscular Atrophy, Spinal genetics
Muscular Atrophy, Spinal pathology
Muscular Dystrophies genetics
Muscular Dystrophies metabolism
Muscular Dystrophies pathology
Mutation
Osteochondrodysplasias congenital
Osteochondrodysplasias genetics
Osteochondrodysplasias metabolism
Osteochondrodysplasias pathology
Parkinson Disease genetics
Parkinson Disease metabolism
Parkinson Disease pathology
Primary Immunodeficiency Diseases
RNA Stability
Ribonucleoproteins analysis
Ribonucleoproteins genetics
Scoliosis genetics
Scoliosis metabolism
Scoliosis pathology
Walker-Warburg Syndrome genetics
Walker-Warburg Syndrome metabolism
Walker-Warburg Syndrome pathology
Dyskeratosis Congenita metabolism
Muscular Atrophy, Spinal metabolism
Ribonucleoproteins metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1471-499X
- Volume :
- 22
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Trends in molecular medicine
- Publication Type :
- Academic Journal
- Accession number :
- 27263464
- Full Text :
- https://doi.org/10.1016/j.molmed.2016.05.005