散发性和综合征性牙源性角化囊肿中 Sonic Hedgehog 信号通路蛋白（Shh、Ptch1、Ptch2、Smo、Gli1、Gli2 和 Gli3）的免疫组织化学评估。
Immunohistochemical evaluation of Sonic Hedgehog signaling pathway proteins (Shh, Ptch1, Ptch2, Smo, Gli1, Gli2, and Gli3) in sporadic and syndromic odontogenic keratocysts.
机构信息
Department of Stomatology, School of Dentistry, University of Sao Paulo, Av. Prof. Lineu Prestes 2227 (Butantã), São Paulo, 03178-200, Brazil.
Department of Bioscience and Oral Diagnosis, Institute of Science and Technology, Paulista State University "Julio de Mesquita Filho" - UNESP, São José dos Campos, São Paulo, Brazil.
出版信息
Clin Oral Investig. 2019 Jan;23(1):153-159. doi: 10.1007/s00784-018-2421-2. Epub 2018 Mar 21.
AIMS
The aim of this study was to compare the clinical and demographic features of 62 patients presenting sporadic odontogenic keratocysts (OKCs) or OKCs associated with nevoid basal cell carcinoma syndrome (NBCCS). In conjunction with this, we also evaluated the immunohistochemical expression of Shh, Ptch1, Ptch2, Smo, Gli1, Gli2 and Gli3 proteins in 86 OKCs. By doing this, we add to the understanding of the biology of this type of lesion, providing tools that will help facilitate the early diagnosis of NBCCS in those patients where the first manifestation is that of OKCs.
METHODS
This is a retrospective study; patients were classified into two groups: group 1 which consisted of those who were not affected by NBCCS (49 patients and 57 OKCs) and group 2 which consisted of those who were diagnosed with NBCCS (13 patients and 29 OKCs). The clinical and demographic features were studied and the immunohistochemical expression of Sonic Hedgehog proteins (Shh, Ptch1, Ptch2, Smo, Gli1, Gli2, and Gli3) was analyzed in all samples.
RESULTS
There was an increase in the expression of three proteins in the syndromic OKC, when compared to that of sporadic cysts. Shh and Gli1 showed higher cytoplasmic expression, while Smo revealed stronger nuclear and cytoplasmic expressions.
CONCLUSION AND CLINICAL RELEVANCE
Our findings suggest that the expression patterns of important Shh pathway proteins can represent valuable markers for early diagnosis of NBCCS-associated OKCs, as the major criterion for the diagnosis of NBCCS is currently based on the late appearance of basal cellular carcinomas. Thus, standardizing a new diagnostic tool for diagnosis of NBCCS could be of great importance in the identification of therapeutic targets. We therefore suggest, as based on our findings, that OKCs showing high expression of Shh, Smo, and Gli1 are potentially associated with NBCCS.
目的
本研究旨在比较 62 例散发牙源性角化囊肿（OKC）或与神经嵴基底细胞癌综合征（NBCCS）相关的 OKC 患者的临床和人口统计学特征。同时，我们还评估了 86 例 OKC 中 Shh、Ptch1、Ptch2、Smo、Gli1、Gli2 和 Gli3 蛋白的免疫组织化学表达。通过这样做，我们增加了对这种病变生物学的理解，提供了有助于早期诊断那些首次表现为 OKC 的 NBCCS 患者的工具。
方法
这是一项回顾性研究；患者被分为两组：组 1 为未受 NBCCS 影响的患者（49 例，57 例 OKC），组 2 为诊断为 NBCCS 的患者（13 例，29 例 OKC）。研究了临床和人口统计学特征，并分析了所有样本中 Sonic Hedgehog 蛋白（Shh、Ptch1、Ptch2、Smo、Gli1、Gli2 和 Gli3）的免疫组织化学表达。
结果
与散发囊肿相比，综合征性 OKC 中三种蛋白的表达增加。Shh 和 Gli1 显示出更高的细胞质表达，而 Smo 则显示出更强的核和细胞质表达。
结论和临床相关性
我们的发现表明，重要的 Shh 通路蛋白的表达模式可能代表 NBCCS 相关 OKC 早期诊断的有价值标志物，因为 NBCCS 的主要诊断标准目前基于基底细胞癌的晚期出现。因此，标准化用于诊断 NBCCS 的新诊断工具对于识别治疗靶点可能非常重要。因此，根据我们的发现，我们建议显示 Shh、Smo 和 Gli1 高表达的 OKC 可能与 NBCCS 相关。
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