Current Understanding of the Role of MicroRNAs in Spinocerebellar Ataxias

Overview

Journal Cerebellum Ataxias

Publisher Biomed Central

Date 2015 Sep 3

PMID 26331031

Citations 10

Authors

Edyta Koscianska

Wlodzimierz J Krzyzosiak

Affiliations

Soon will be listed here.

Abstract

The number of studies highlighting the role of microRNAs (miRNAs) in human physiology and diseases is growing, but many miRNA-driven regulatory mechanisms remain elusive. A proper understanding of the exact functions of individual miRNAs and their interaction with specific targets is vitally important because such knowledge might help cure diseases for which no effective treatment currently exists. Herein, we present current views on the role of the miRNA-mediated regulation of gene expression in the case of select spinocerebellar ataxias (SCAs) and their potential involvement in the pathogenesis of these diseases. Specifically, we summarize published data showing the known links between miRNAs and CAG repeat-dependent SCAs. Moreover, using the example of SCA type 3 (SCA3), we refer to the issue of prediction and validation of miRNA targets, and we demonstrate that miR-181a-1 may regulate the 3'-UTR of the ATXN3 gene.

Citing Articles

Regulatory Potential of Competing Endogenous RNAs in Myotonic Dystrophies.

Koscianska E, Kozlowska E, Fiszer A Int J Mol Sci. 2021; 22(11).

PMID: 34200099 PMC: 8201210. DOI: 10.3390/ijms22116089.

miR-150 is a negative independent prognostic biomarker for primary gastrointestinal diffuse large B-cell lymphoma.

Wang X, Kan Y, Chen L, Ge P, Ding T, Zhai Q Oncol Lett. 2020; 19(5):3487-3494.

PMID: 32269622 PMC: 7115130. DOI: 10.3892/ol.2020.11452.

Role of Nuclear Factor Kappa B (NF-κB) Signalling in Neurodegenerative Diseases: An Mechanistic Approach.

Singh S, Singh T Curr Neuropharmacol. 2020; 18(10):918-935.

PMID: 32031074 PMC: 7709146. DOI: 10.2174/1570159X18666200207120949.

Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7.

Niewiadomska-Cimicka A, Trottier Y Neurotherapeutics. 2019; 16(4):1074-1096.

PMID: 31432449 PMC: 6985300. DOI: 10.1007/s13311-019-00778-5.

The Emerging Role of microRNAs in Polyglutamine Diseases.

Dong X, Cong S Front Mol Neurosci. 2019; 12:156.

PMID: 31275113 PMC: 6593396. DOI: 10.3389/fnmol.2019.00156.

References

Cemal C, Carroll C, Lawrence L, Lowrie M, Ruddle P, Al-Mahdawi S . YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit. Hum Mol Genet. 2002; 11(9):1075-94. DOI: 10.1093/hmg/11.9.1075. View

Krol J, Loedige I, Filipowicz W . The widespread regulation of microRNA biogenesis, function and decay. Nat Rev Genet. 2010; 11(9):597-610. DOI: 10.1038/nrg2843. View

Burright E, Clark H, Servadio A, Matilla T, Feddersen R, Yunis W . SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 1995; 82(6):937-48. DOI: 10.1016/0092-8674(95)90273-2. View

Kuhn D, Martin M, Feldman D, Terry Jr A, Nuovo G, Elton T . Experimental validation of miRNA targets. Methods. 2007; 44(1):47-54. PMC: 2237914. DOI: 10.1016/j.ymeth.2007.09.005. View

Reinhardt A, Feuillette S, Cassar M, Callens C, Thomassin H, Birman S . Lack of miRNA Misregulation at Early Pathological Stages in Drosophila Neurodegenerative Disease Models. Front Genet. 2012; 3:226. PMC: 3483601. DOI: 10.3389/fgene.2012.00226. View

Sinha M, Ghose J, Das E, Bhattarcharyya N . Altered microRNAs in STHdh(Q111)/Hdh(Q111) cells: miR-146a targets TBP. Biochem Biophys Res Commun. 2010; 396(3):742-7. DOI: 10.1016/j.bbrc.2010.05.007. View

Rodriguez-Lebron E, Liu G, Keiser M, Behlke M, Davidson B . Altered Purkinje cell miRNA expression and SCA1 pathogenesis. Neurobiol Dis. 2013; 54:456-63. PMC: 3629010. DOI: 10.1016/j.nbd.2013.01.019. View

Fiszer A, Krzyzosiak W . Oligonucleotide-based strategies to combat polyglutamine diseases. Nucleic Acids Res. 2014; 42(11):6787-810. PMC: 4066792. DOI: 10.1093/nar/gku385. View

Delay C, Mandemakers W, Hebert S . MicroRNAs in Alzheimer's disease. Neurobiol Dis. 2012; 46(2):285-90. DOI: 10.1016/j.nbd.2012.01.003. View

10.

Ranum L, Lundgren J, Schut L, Ahrens M, Perlman S, Aita J . Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia. Am J Hum Genet. 1995; 57(3):603-8. PMC: 1801263. View

11.

Shi Y, Huang F, Tang B, Li J, Wang J, Shen L . MicroRNA profiling in the serums of SCA3/MJD patients. Int J Neurosci. 2013; 124(2):97-101. DOI: 10.3109/00207454.2013.827679. View

12.

Zhuchenko O, Bailey J, Bonnen P, Ashizawa T, Stockton D, Amos C . Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel. Nat Genet. 1997; 15(1):62-9. DOI: 10.1038/ng0197-62. View

13.

Kim V, Han J, Siomi M . Biogenesis of small RNAs in animals. Nat Rev Mol Cell Biol. 2009; 10(2):126-39. DOI: 10.1038/nrm2632. View

14.

Persengiev S, Kondova I, Otting N, Koeppen A, Bontrop R . Genome-wide analysis of miRNA expression reveals a potential role for miR-144 in brain aging and spinocerebellar ataxia pathogenesis. Neurobiol Aging. 2010; 32(12):2316.e17-27. DOI: 10.1016/j.neurobiolaging.2010.03.014. View

15.

Lewis B, Burge C, Bartel D . Conserved seed pairing, often flanked by adenosines, indicates that thousands of human genes are microRNA targets. Cell. 2005; 120(1):15-20. DOI: 10.1016/j.cell.2004.12.035. View

16.

Zoghbi H, Orr H . Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1. J Biol Chem. 2008; 284(12):7425-9. PMC: 2658037. DOI: 10.1074/jbc.R800041200. View

17.

David G, Abbas N, Stevanin G, Durr A, Yvert G, Cancel G . Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion. Nat Genet. 1997; 17(1):65-70. DOI: 10.1038/ng0997-65. View

18.

Gehrke S, Imai Y, Sokol N, Lu B . Pathogenic LRRK2 negatively regulates microRNA-mediated translational repression. Nature. 2010; 466(7306):637-41. PMC: 3049892. DOI: 10.1038/nature09191. View

19.

Sun A, Crabtree G, Yoo A . MicroRNAs: regulators of neuronal fate. Curr Opin Cell Biol. 2013; 25(2):215-21. PMC: 3836262. DOI: 10.1016/j.ceb.2012.12.007. View

20.

Eacker S, Dawson T, Dawson V . The interplay of microRNA and neuronal activity in health and disease. Front Cell Neurosci. 2013; 7:136. PMC: 3753455. DOI: 10.3389/fncel.2013.00136. View