Insights and future directions of potential genetic therapy for Apert syndrome: A systematic review

被引:5
作者
Al-Namnam, Nisreen Mohammed [1 ]
Jayash, Soher Nagi [2 ]
Hariri, Firdaus [3 ]
Rahman, Zainal Ariff Abdul [3 ]
Alshawsh, Mohammed Abdullah [4 ]
机构
[1] Univ Sanaa, Dept Oral Surg, Fac Dent, Sanaa, Yemen
[2] Univ Birmingham, Sch Dent, 5 Mill Pool Way, Birmingham, W Midlands, England
[3] Univ Malaya, Dept Oral & Maxillofacial Clin Sci, Fac Dent, Kuala Lumpur 50603, Malaysia
[4] Univ Malaya, Dept Pharmacol, Fac Med, Kuala Lumpur 50603, Malaysia
关键词
FACTOR RECEPTOR-2 FGFR2; ORAL MANIFESTATIONS; SOLUBLE FORM; MUTATIONS; CRANIOSYNOSTOSIS; ACTIVATION; INHIBITION; PHENOTYPES;
D O I
10.1038/s41434-021-00238-w
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Apert syndrome is a genetic disorder characterised by craniosynostosis and structural discrepancy of the craniofacial region as well as the hands and feet. This condition is closely linked with fibroblast growth factor receptor-2 (FGFR2) gene mutations. Gene therapies are progressively being tested in advanced clinical trials, leading to a rise of its potential clinical indications. In recent years, research has made great progress in the gene therapy of craniosynostosis syndromes and several studies have investigated its influences in preventing/diminishing the complications of Apert syndrome. This article reviewed and exhibited different techniques of gene therapy and their influences in Apert syndrome progression. A systematic search was executed using electronic bibliographic databases including PubMed, EMBASE, ScienceDirect, SciFinder and Web of Science for all studies of gene therapy for Apert syndrome. The primary outcomes measurements vary from protein to gene expressions. According to the findings of included studies, we conclude that the gene therapy using FGF in Apert syndrome was critical in the regulation of suture fusion and patency, occurred via alterations in cellular proliferation. The superior outcome could be brought by biological therapies targeting the FGF/FGFR signalling. More studies in molecular genetics in Apert syndrome are recommended. This study reviews the current literature and provides insights to future possibilities of genetic therapy as intervention in Apert syndrome.
引用
收藏
页码:620 / 633
页数:14
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