Molecular neurobiology, 2021-02, Vol.58 (2), p.631-646
2021
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- Autor(en) / Beteiligte
- Titel
- Heterogeneous Nuclear Ribonucleoproteins: Implications in Neurological Diseases
- Ist Teil von
- Molecular neurobiology, 2021-02, Vol.58 (2), p.631-646
- Ort / Verlag
- New York: Springer US
- Erscheinungsjahr
- 2021
- Quelle
- SpringerLink
- Beschreibungen/Notizen
- Heterogenous nuclear ribonucleoproteins (hnRNPs) are a complex and functionally diverse family of RNA binding proteins with multifarious roles. They are involved, directly or indirectly, in alternative splicing, transcriptional and translational regulation, stress granule formation, cell cycle regulation, and axonal transport. It is unsurprising, given their heavy involvement in maintaining functional integrity of the cell, that their dysfunction has neurological implications. However, compared to their more established roles in cancer, the evidence of hnRNP implication in neurological diseases is still in its infancy. This review aims to consolidate the evidences for hnRNP involvement in neurological diseases, with a focus on spinal muscular atrophy (SMA), Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), multiple sclerosis (MS), congenital myasthenic syndrome (CMS), and fragile X-associated tremor/ataxia syndrome (FXTAS). Understanding more about hnRNP involvement in neurological diseases can further elucidate the pathomechanisms involved in these diseases and perhaps guide future therapeutic advances.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0893-7648, 1559-1182eISSN: 1559-1182DOI: 10.1007/s12035-020-02137-4Titel-ID: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_7843550
- Format
- –
- Schlagworte
- Alternative splicing, Alternative Splicing - genetics, Alzheimer's disease, Amyotrophic lateral sclerosis, Animals, Ataxia, Axonal Transport, Biomedical and Life Sciences, Biomedicine, Cell Biology, Cell cycle, Dementia disorders, Fragile X syndrome, Frontotemporal dementia, Gene regulation, Heterogeneous-Nuclear Ribonucleoproteins - chemistry, Heterogeneous-Nuclear Ribonucleoproteins - genetics, Heterogeneous-Nuclear Ribonucleoproteins - metabolism, Humans, Intellectual disabilities, Models, Biological, Multiple sclerosis, Nervous System Diseases - metabolism, Neurobiology, Neurodegenerative diseases, Neurological diseases, Neurology, Neuromuscular diseases, Neurosciences, Ribonucleic acid, Ribonucleoproteins, RNA, RNA-binding protein, Spinal muscular atrophy, Transcription, Tremor