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疑似家族性腺瘤性息肉病(FAP)家系中APC基因第15外显子突变的鉴定。

Identification of APC exon 15 mutations in families suspected of familial adenomatous polyposis (FAP).

作者信息

Kirchhoff T, Zajac V, Krizan P, Repiská V, Stevurková V, Friedl W

机构信息

Cancer Research Institute, Slovak Academy of Sciences, Bratislava, Slovakia.

出版信息

Folia Biol (Praha). 1997;43(5):203-9.

PMID:9595262
Abstract

Patients with familial adenomatous polyposis coli (FAP) reveal numerous colorectal adenomas as well as benign and malignant extracolonic lesions. Adenomatous polyposis coli (APC) gene mutations are the crucial genetic defect in FAP. The APC mutation molecular analysis of 20 FAP families was performed using the novel and effective method of the heteroduplex analysis (HDA). All of these families were screened for mutations in APC exon 15. APC mutations were identified in 4 individuals of two families. These two families were also screened by the protein truncation test (PTT). The PTT results confirmed previous findings obtained by HDA. The results of molecular analysis were correlated with the clinical manifestations of extracolonic lesions and congenital hypertrophy of retinal pigment epithelium (CHRPE). Positive correlation of all clinical examinations and mutations of APC gene was observed in all 4 FAP patients.

摘要

家族性腺瘤性息肉病(FAP)患者会出现大量结直肠腺瘤以及良性和恶性的结肠外病变。腺瘤性息肉病(APC)基因突变是FAP的关键遗传缺陷。采用新型有效方法——异源双链分析(HDA)对20个FAP家族进行了APC突变分子分析。对所有这些家族的APC外显子15进行了突变筛查。在两个家族的4名个体中发现了APC突变。这两个家族也通过蛋白质截短试验(PTT)进行了筛查。PTT结果证实了先前通过HDA获得的结果。分子分析结果与结肠外病变和视网膜色素上皮先天性肥大(CHRPE)的临床表现相关。在所有4名FAP患者中均观察到所有临床检查与APC基因突变呈正相关。

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