G-Quadruplexes in Repeat Expansion Disorders
The repeat expansions are the main genetic cause of various neurodegeneration diseases. More than ten kinds of repeat sequences with different lengths, locations, and structures have been confirmed in the past two decades. G-rich repeat sequences, such as CGG and GGGGCC, are reported to form functio...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-01-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/24/3/2375 |
| _version_ | 1797624437730705408 |
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| author | Ye Teng Ming Zhu Zhidong Qiu |
| author_facet | Ye Teng Ming Zhu Zhidong Qiu |
| author_sort | Ye Teng |
| collection | DOAJ |
| description | The repeat expansions are the main genetic cause of various neurodegeneration diseases. More than ten kinds of repeat sequences with different lengths, locations, and structures have been confirmed in the past two decades. G-rich repeat sequences, such as CGG and GGGGCC, are reported to form functional G-quadruplexes, participating in many important bioprocesses. In this review, we conducted an overview concerning the contribution of G-quadruplex in repeat expansion disorders and summarized related mechanisms in current pathological studies, including the increasing genetic instabilities in replication and transcription, the toxic RNA foci formed in neurons, and the loss/gain function of proteins and peptides. Furthermore, novel strategies targeting G-quadruplex repeats were developed based on the understanding of disease mechanism. Small molecules and proteins binding to G-quadruplex in repeat expansions were investigated to protect neurons from dysfunction and delay the progression of neurodegeneration. In addition, the effects of environment on the stability of G-quadruplex were discussed, which might be critical factors in the pathological study of repeat expansion disorders. |
| first_indexed | 2024-03-11T09:42:21Z |
| format | Article |
| id | doaj.art-ebc971d62c134f238be81670a8622ee5 |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-11T09:42:21Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-ebc971d62c134f238be81670a8622ee52023-11-16T16:56:21ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672023-01-01243237510.3390/ijms24032375G-Quadruplexes in Repeat Expansion DisordersYe Teng0Ming Zhu1Zhidong Qiu2School of Pharmacy, Changchun University of Chinese Medicine, 1035 Boshuo Road, Changchun 130117, ChinaSchool of Pharmacy, Changchun University of Chinese Medicine, 1035 Boshuo Road, Changchun 130117, ChinaSchool of Pharmacy, Changchun University of Chinese Medicine, 1035 Boshuo Road, Changchun 130117, ChinaThe repeat expansions are the main genetic cause of various neurodegeneration diseases. More than ten kinds of repeat sequences with different lengths, locations, and structures have been confirmed in the past two decades. G-rich repeat sequences, such as CGG and GGGGCC, are reported to form functional G-quadruplexes, participating in many important bioprocesses. In this review, we conducted an overview concerning the contribution of G-quadruplex in repeat expansion disorders and summarized related mechanisms in current pathological studies, including the increasing genetic instabilities in replication and transcription, the toxic RNA foci formed in neurons, and the loss/gain function of proteins and peptides. Furthermore, novel strategies targeting G-quadruplex repeats were developed based on the understanding of disease mechanism. Small molecules and proteins binding to G-quadruplex in repeat expansions were investigated to protect neurons from dysfunction and delay the progression of neurodegeneration. In addition, the effects of environment on the stability of G-quadruplex were discussed, which might be critical factors in the pathological study of repeat expansion disorders.https://www.mdpi.com/1422-0067/24/3/2375G-quadruplexrepeat expansion disorderneurodegenerationRNA foci |
| spellingShingle | Ye Teng Ming Zhu Zhidong Qiu G-Quadruplexes in Repeat Expansion Disorders International Journal of Molecular Sciences G-quadruplex repeat expansion disorder neurodegeneration RNA foci |
| title | G-Quadruplexes in Repeat Expansion Disorders |
| title_full | G-Quadruplexes in Repeat Expansion Disorders |
| title_fullStr | G-Quadruplexes in Repeat Expansion Disorders |
| title_full_unstemmed | G-Quadruplexes in Repeat Expansion Disorders |
| title_short | G-Quadruplexes in Repeat Expansion Disorders |
| title_sort | g quadruplexes in repeat expansion disorders |
| topic | G-quadruplex repeat expansion disorder neurodegeneration RNA foci |
| url | https://www.mdpi.com/1422-0067/24/3/2375 |
| work_keys_str_mv | AT yeteng gquadruplexesinrepeatexpansiondisorders AT mingzhu gquadruplexesinrepeatexpansiondisorders AT zhidongqiu gquadruplexesinrepeatexpansiondisorders |