Journal
MOLECULAR NEUROBIOLOGY
Volume 58, Issue 2, Pages 631-646Publisher
SPRINGER
DOI: 10.1007/s12035-020-02137-4
Keywords
hnRNPs; Alzheimer's disease; Multiple sclerosis; ALS; FTD
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Funding
- Alzheimer's Research UK senior fellowship
- Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology
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Heterogenous nuclear ribonucleoproteins (hnRNPs) play diverse roles in RNA regulation and have implications in neurological diseases, although evidence is still emerging. Understanding their involvement in neurological diseases can shed light on pathomechanisms and potentially advance therapeutic interventions.
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.
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