Home > Publications Database > The MID1 Protein: A Promising Therapeutic Target in Huntington's Disease. > print

001     162830
005     20230915092409.0
024 7 _ |a 10.3389/fgene.2021.761714
|2 doi
024 7 _ |a pmid:34659371
|2 pmid
024 7 _ |a pmc:PMC8517220
|2 pmc
024 7 _ |a altmetric:114312454
|2 altmetric
037 _ _ |a DZNE-2021-01485
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Heinz, Annika
|b 0
245 _ _ |a The MID1 Protein: A Promising Therapeutic Target in Huntington's Disease.
260 _ _ |a Lausanne
|c 2021
|b Frontiers Media
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 1655472191_22304
|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
500 _ _ |a (CC BY)
520 _ _ |a Huntington's disease (HD) is caused by an expansion mutation of a CAG repeat in exon 1 of the huntingtin (HTT) gene, that encodes an expanded polyglutamine tract in the HTT protein. HD is characterized by progressive psychiatric and cognitive symptoms associated with a progressive movement disorder. HTT is ubiquitously expressed, but the pathological changes caused by the mutation are most prominent in the central nervous system. Since the mutation was discovered, research has mainly focused on the mutant HTT protein. But what if the polyglutamine protein is not the only cause of the neurotoxicity? Recent studies show that the mutant RNA transcript is also involved in cellular dysfunction. Here we discuss the abnormal interaction of the mutant HTT transcript with a protein complex containing the MID1 protein. MID1 aberrantly binds to CAG repeats and this binding increases with CAG repeat length. Since MID1 is a translation regulator, association of the MID1 complex stimulates translation of mutant HTT mRNA, resulting in an overproduction of polyglutamine protein. Thus, blocking the interaction between MID1 and mutant HTT mRNA is a promising therapeutic approach. Additionally, we show that MID1 expression in the brain of both HD patients and HD mice is aberrantly increased. This finding further supports the concept of blocking the interaction between MID1 and mutant HTT mRNA to counteract mutant HTT translation as a valuable therapeutic strategy. In line, recent studies in which either compounds affecting the assembly of the MID1 complex or molecules targeting HTT RNA, show promising results.
536 _ _ |a 352 - Disease Mechanisms (POF4-352)
|0 G:(DE-HGF)POF4-352
|c POF4-352
|f POF IV
|x 0
588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
650 _ 7 |a Alzheimer’s disease
|2 Other
650 _ 7 |a CAG repeat
|2 Other
650 _ 7 |a Huntington’s disease
|2 Other
650 _ 7 |a MID1
|2 Other
650 _ 7 |a RNA-protein interaction
|2 Other
650 _ 7 |a RNA-targeting drug
|2 Other
650 _ 7 |a mRNA translation
|2 Other
700 1 _ |a Schilling, Judith
|0 P:(DE-2719)2810643
|b 1
700 1 _ |a van Roon-Mom, Willeke
|b 2
700 1 _ |a Krauß, Sybille
|0 P:(DE-HGF)0
|b 3
773 _ _ |a 10.3389/fgene.2021.761714
|g Vol. 12, p. 761714
|0 PERI:(DE-600)2606823-0
|p 761714
|t Frontiers in genetics
|v 12
|y 2021
|x 1664-8021
856 4 _ |y OpenAccess
|u https://pub.dzne.de/record/162830/files/DZNE-2021-01485.pdf
856 4 _ |y OpenAccess
|x pdfa
|u https://pub.dzne.de/record/162830/files/DZNE-2021-01485.pdf?subformat=pdfa
909 C O |o oai:pub.dzne.de:162830
|p openaire
|p open_access
|p VDB
|p driver
|p dnbdelivery
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 1
|6 P:(DE-2719)2810643
913 1 _ |a DE-HGF
|b Gesundheit
|l Neurodegenerative Diseases
|1 G:(DE-HGF)POF4-350
|0 G:(DE-HGF)POF4-352
|3 G:(DE-HGF)POF4
|2 G:(DE-HGF)POF4-300
|4 G:(DE-HGF)POF
|v Disease Mechanisms
|x 0
914 1 _ |y 2021
915 _ _ |a Creative Commons Attribution CC BY (No Version)
|0 LIC:(DE-HGF)CCBYNV
|2 V:(DE-HGF)
|b DOAJ
|d 2021-05-04
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)1190
|2 StatID
|b Biological Abstracts
|d 2021-05-04
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0160
|2 StatID
|b Essential Science Indicators
|d 2021-05-04
915 _ _ |a WoS
|0 StatID:(DE-HGF)0113
|2 StatID
|b Science Citation Index Expanded
|d 2021-05-04
915 _ _ |a Fees
|0 StatID:(DE-HGF)0700
|2 StatID
|d 2021-05-04
915 _ _ |a OpenAccess
|0 StatID:(DE-HGF)0510
|2 StatID
915 _ _ |a Article Processing Charges
|0 StatID:(DE-HGF)0561
|2 StatID
|d 2021-05-04
915 _ _ |a JCR
|0 StatID:(DE-HGF)0100
|2 StatID
|b FRONT GENET : 2021
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0200
|2 StatID
|b SCOPUS
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0300
|2 StatID
|b Medline
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0501
|2 StatID
|b DOAJ Seal
|d 2021-05-04T10:15:44Z
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0500
|2 StatID
|b DOAJ
|d 2021-05-04T10:15:44Z
915 _ _ |a Peer Review
|0 StatID:(DE-HGF)0030
|2 StatID
|b DOAJ : Blind peer review
|d 2021-05-04T10:15:44Z
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0199
|2 StatID
|b Clarivate Analytics Master Journal List
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0150
|2 StatID
|b Web of Science Core Collection
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)1050
|2 StatID
|b BIOSIS Previews
|d 2022-11-08
915 _ _ |a IF < 5
|0 StatID:(DE-HGF)9900
|2 StatID
|d 2022-11-08
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 UNRESTRICTED
980 _ _ |a I:(DE-2719)1011006
980 1 _ |a FullTexts

LibraryCollectionCLSMajorCLSMinorLanguageAuthor
Marc 21