001     141139
005     20240321220929.0
024 7 _ |a 10.2174/2211536607666180821162403
|2 doi
024 7 _ |a pmid:30147021
|2 pmid
024 7 _ |a 2211-5366
|2 ISSN
024 7 _ |a 2211-5374
|2 ISSN
037 _ _ |a DZNE-2020-07461
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Krauß, Sybille
|0 P:(DE-2719)2421562
|b 0
|e First author
245 _ _ |a Upregulation of miR-25 and miR-181 Family Members Correlates with Reduced Expression of ATXN3 in Lymphocytes from SCA3 Patients.
260 _ _ |a Sharjah [u.a.]
|c 2019
|b Bentham Science
264 _ 1 |3 print
|2 Crossref
|b Bentham Science Publishers Ltd.
|c 2018-11-27
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
|2 DataCite
336 7 _ |a Journal Article
|b journal
|m journal
|0 PUB:(DE-HGF)16
|s 1700147394_27198
|2 PUB:(DE-HGF)
336 7 _ |a ARTICLE
|2 BibTeX
336 7 _ |a JOURNAL_ARTICLE
|2 ORCID
336 7 _ |a Journal Article
|0 0
|2 EndNote
520 _ _ |a Spinocerebellar ataxia type 3 (SCA3), the most common spinocerebellar ataxia, is caused by a polyglutamine (polyQ) expansion in the protein ataxin-3 (ATXN3). Silencing the expression of polyQ-expanded ATXN3 rescues the cellular disease phenotype.This study investigated the differential expression of microRNAs (miRNAs), small noncoding RNAs targeting gene expression, in lymphoblastoid cells (LCs) from SCA3 patients and the capability of identified deregulated miRNAs to target and alter ATXN3 expression.MiRNA profiling was performed by microarray hybridization of total RNA from control and SCA3-LCs. The capability of the identified miRNAs and their target sites to suppress ATXN3 expression was analyzed using mutagenesis, reverse transcription PCR, immunoblotting, luciferase reporter assays, mimics and precursors of the identified miRNAs.SCA3-LCs showed significantly decreased expression levels of ATXN3 and a significant upregulation of the ATXN3-3'UTR targeting miRNAs, miR-32 and miR-181c and closely related members of the miR-25 and miR-181 family, respectively. MiR-32 and miR-181c effectively targeted the 3'UTR of ATXN3 and suppressed the expression of ATXN3.The simultaneous upregulation of closely related miRNAs targeting the 3'UTR of ATXN3 and the significantly reduced ATXN3 expression levels in SCA3-LCs suggests that miR-25 and miR-181 family members cooperatively bind to the 3'UTR to suppress the expression of ATXN3. The findings further suggest that the upregulation of miR-25 and miR-181 family members in SCA3- LCs reflects a cell type-specific, protective mechanism to diminish polyQ-mediated cytotoxic effects. Thus, miRNA mimics of miR-25 and miR-181 family members may prove useful for the treatment of SCA3.
536 _ _ |a 342 - Disease Mechanisms and Model Systems (POF3-342)
|0 G:(DE-HGF)POF3-342
|c POF3-342
|f POF III
|x 0
542 _ _ |i 2018-11-27
|2 Crossref
|u https://creativecommons.org/licenses/by/4.0/legalcode
588 _ _ |a Dataset connected to CrossRef, PubMed,
650 _ 7 |a 3' Untranslated Regions
|2 NLM Chemicals
650 _ 7 |a MIRN25 microRNA, human
|2 NLM Chemicals
650 _ 7 |a MIrn181 microRNA, human
|2 NLM Chemicals
650 _ 7 |a MicroRNAs
|2 NLM Chemicals
650 _ 7 |a Repressor Proteins
|2 NLM Chemicals
650 _ 7 |a ATXN3 protein, human
|0 EC 3.4.19.12
|2 NLM Chemicals
650 _ 7 |a Ataxin-3
|0 EC 3.4.19.12
|2 NLM Chemicals
650 _ 2 |a 3' Untranslated Regions
|2 MeSH
650 _ 2 |a Ataxin-3: genetics
|2 MeSH
650 _ 2 |a Ataxin-3: metabolism
|2 MeSH
650 _ 2 |a HeLa Cells
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Lymphocytes: metabolism
|2 MeSH
650 _ 2 |a Machado-Joseph Disease: genetics
|2 MeSH
650 _ 2 |a Machado-Joseph Disease: metabolism
|2 MeSH
650 _ 2 |a MicroRNAs: genetics
|2 MeSH
650 _ 2 |a MicroRNAs: metabolism
|2 MeSH
650 _ 2 |a Repressor Proteins: genetics
|2 MeSH
650 _ 2 |a Repressor Proteins: metabolism
|2 MeSH
700 1 _ |a Nalavade, Rohit
|0 P:(DE-2719)2809904
|b 1
700 1 _ |a Weber, Stephanie
|0 P:(DE-2719)2662310
|b 2
700 1 _ |a Carter, Katlynn
|0 P:(DE-HGF)0
|b 3
700 1 _ |a Evert, Bernd O
|0 P:(DE-HGF)0
|b 4
773 1 8 |a 10.2174/2211536607666180821162403
|b : Bentham Science Publishers Ltd., 2018-11-27
|n 1
|p 76-85
|3 journal-article
|2 Crossref
|t MicroRNA
|v 8
|y 2018
|x 2211-5366
773 _ _ |a 10.2174/2211536607666180821162403
|g Vol. 8, no. 1, p. 76 - 85
|0 PERI:(DE-600)2800970-8
|n 1
|q 8:1<76 - 85
|p 76-85
|t MicroRNA
|v 8
|y 2019
|x 2211-5366
856 4 _ |u https://pub.dzne.de/record/141139/files/DZNE-2020-07461_Restricted.pdf
856 4 _ |u https://pub.dzne.de/record/141139/files/DZNE-2020-07461_Restricted.pdf?subformat=pdfa
|x pdfa
909 C O |p VDB
|o oai:pub.dzne.de:141139
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 0
|6 P:(DE-2719)2421562
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 1
|6 P:(DE-2719)2809904
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 2
|6 P:(DE-2719)2662310
913 1 _ |a DE-HGF
|b Gesundheit
|l Erkrankungen des Nervensystems
|1 G:(DE-HGF)POF3-340
|0 G:(DE-HGF)POF3-342
|3 G:(DE-HGF)POF3
|2 G:(DE-HGF)POF3-300
|4 G:(DE-HGF)POF
|v Disease Mechanisms and Model Systems
|x 0
914 1 _ |y 2019
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0200
|2 StatID
|b SCOPUS
|d 2022-11-19
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0300
|2 StatID
|b Medline
|d 2022-11-19
920 1 _ |0 I:(DE-2719)1011006
|k AG Krauß
|l Regulatory RNA-protein interaction in neurodegenerative diseases
|x 0
980 _ _ |a journal
980 _ _ |a VDB
980 _ _ |a I:(DE-2719)1011006
980 _ _ |a UNRESTRICTED
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LibraryCollectionCLSMajorCLSMinorLanguageAuthor
Marc 21