How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway
Fanconi Anemia (FA) is a rare human genetic disorder characterized by developmental defects, bone marrow failure and cancer predisposition, primarily due to a deficiency in the repair of DNA interstrand crosslinks (ICLs). ICL repair through the FA DNA repair pathway is a complicated multi-step proce...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2016-04-01
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| Series: | Frontiers in Genetics |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/Journal/10.3389/fgene.2016.00061/full |
| _version_ | 1819259657807462400 |
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| author | Kate eColeman Tony T Huang |
| author_facet | Kate eColeman Tony T Huang |
| author_sort | Kate eColeman |
| collection | DOAJ |
| description | Fanconi Anemia (FA) is a rare human genetic disorder characterized by developmental defects, bone marrow failure and cancer predisposition, primarily due to a deficiency in the repair of DNA interstrand crosslinks (ICLs). ICL repair through the FA DNA repair pathway is a complicated multi-step process, involving at least 19 FANC proteins and coordination of multiple DNA repair activities, including homologous recombination (HR), nucleotide excision repair (NER) and translesion synthesis (TLS). SUMOylation is a critical regulator of several DNA repair pathways, however, the role of this modification in controlling the FA pathway is poorly understood. Here, we summarize recent advances in the fine-tuning of the FA pathway by SUMO-targeted ubiquitin ligases (STUbLs) and other SUMO-related interactions, and discuss the implications of these findings in the design of novel therapeutics for alleviating FA-associated condition, including cancer. |
| first_indexed | 2024-12-23T19:13:30Z |
| format | Article |
| id | doaj.art-9d69a836374a4cc1b5c131a29e584d70 |
| institution | Directory Open Access Journal |
| issn | 1664-8021 |
| language | English |
| last_indexed | 2024-12-23T19:13:30Z |
| publishDate | 2016-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Genetics |
| spelling | doaj.art-9d69a836374a4cc1b5c131a29e584d702022-12-21T17:34:22ZengFrontiers Media S.A.Frontiers in Genetics1664-80212016-04-01710.3389/fgene.2016.00061195945How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair PathwayKate eColeman0Tony T Huang1New York University School of MedicineNew York University School of MedicineFanconi Anemia (FA) is a rare human genetic disorder characterized by developmental defects, bone marrow failure and cancer predisposition, primarily due to a deficiency in the repair of DNA interstrand crosslinks (ICLs). ICL repair through the FA DNA repair pathway is a complicated multi-step process, involving at least 19 FANC proteins and coordination of multiple DNA repair activities, including homologous recombination (HR), nucleotide excision repair (NER) and translesion synthesis (TLS). SUMOylation is a critical regulator of several DNA repair pathways, however, the role of this modification in controlling the FA pathway is poorly understood. Here, we summarize recent advances in the fine-tuning of the FA pathway by SUMO-targeted ubiquitin ligases (STUbLs) and other SUMO-related interactions, and discuss the implications of these findings in the design of novel therapeutics for alleviating FA-associated condition, including cancer.http://journal.frontiersin.org/Journal/10.3389/fgene.2016.00061/fullSumoylationUbiquitinhomologous recombination (HR)deubiquitinases (DUBs)Nucleotide Excision Repair (NER)Translesion synthesis (TLS) |
| spellingShingle | Kate eColeman Tony T Huang How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway Frontiers in Genetics Sumoylation Ubiquitin homologous recombination (HR) deubiquitinases (DUBs) Nucleotide Excision Repair (NER) Translesion synthesis (TLS) |
| title | How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway |
| title_full | How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway |
| title_fullStr | How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway |
| title_full_unstemmed | How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway |
| title_short | How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway |
| title_sort | how sumoylation fine tunes the fanconi anemia dna repair pathway |
| topic | Sumoylation Ubiquitin homologous recombination (HR) deubiquitinases (DUBs) Nucleotide Excision Repair (NER) Translesion synthesis (TLS) |
| url | http://journal.frontiersin.org/Journal/10.3389/fgene.2016.00061/full |
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