Department of Medical Genetics, Iran University of Medical Sciences (IUMS), Tehran, Iran.
Department of Pathology, Tehran University of Medical Sciences (TUMS), Tehran, Iran.
Int J Dermatol. 2018 Dec;57(12):1485-1491. doi: 10.1111/ijd.14213. Epub 2018 Sep 23.
Filaggrin is a key structural epidermal protein in terminal differentiation and formation of skin barrier. The important role of filaggrin and its effects in various cutaneous and noncutaneous disorders initiated a cascade of considerable research in recent years. Loss-of-function mutations in FLG, the human gene encoding profilaggrin/filaggrin, is the cause of the common skin condition ichthyosis vulgaris (IV) and major genetic predisposing factor for atopic dermatitis (AD). Several null mutations in the FLG gene that lead to a decrease or absence of filaggrin in skin and predispose these conditions have been described.
The aim of this study was to investigative genetic polymorphism of FLG in Iranian patients with IV and AD.
In the current study, we carried out full sequencing of the entire FLG coding region in 30 IV patients and 30 AD patients, and also 60 healthy controls.
In our research, we identified 43 variants reported previously and two novel variants.
In our study, in the AD and IV patients, loss-of-function FLG mutation was not found. This means that another mechanism other than FLG nonsense mutation is involved in the pathogenesis of these patients.
丝聚蛋白是终末分化和皮肤屏障形成过程中的关键结构表皮蛋白。丝聚蛋白的重要作用及其在各种皮肤和非皮肤疾病中的影响促使近年来进行了大量的研究。编码前丝聚蛋白/丝聚蛋白的人类基因 FLG 的功能丧失性突变是常见皮肤疾病寻常型鱼鳞病(IV)的病因,也是特应性皮炎(AD)的主要遗传易患因素。已经描述了导致皮肤中丝聚蛋白减少或缺失并易患这些疾病的 FLG 基因中的几个无义突变。
本研究旨在调查伊朗 IV 型和 AD 患者中 FLG 的遗传多态性。
在本研究中,我们对 30 例 IV 型患者、30 例 AD 患者和 60 例健康对照者进行了整个 FLG 编码区的全序列分析。
在我们的研究中,我们鉴定了之前报道的 43 个变体和两个新变体。
在我们的研究中,在 AD 和 IV 患者中,未发现功能丧失性 FLG 突变。这意味着除了 FLG 无义突变之外,还有其他机制参与了这些患者的发病机制。