Distinctive expression patterns of glycoprotein non-metastatic B and folliculin in renal tumors in patients with Birt-Hogg-Dubé syndrome.
Bottom Line: Western blot and immunostaining analyses revealed that FLCN-related RCCs showed overexpression of GPNMB and underexpression of FLCN, whereas sporadic tumors showed inverted patterns.GPNMB mRNA in FLCN-related RCCs was 23-fold more abundant than in sporadic tumors.The distinctive expression patterns of GPNMB and FLCN might identify patients with RCCs who need further work-up for BHD.
Affiliation: Department of Molecular Pathology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.Show MeSH
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Mentions: Western blotting clearly indicated the loss of FLCN, but immunostaining for FLCN was not sufficiently specific to predict RCC associated with BHD. Some auxiliary markers were desirable to give an indication for genetic analysis. In the current study, we compared GPNMB expression between BHD and sporadic tumors. In quantitative RT-PCR, RCCs from BHD patients expressed GPNMB mRNA at levels that averaged 23-fold higher compared to sporadic RCCs (P < 0.01, Student's t-test) (Fig.5a). GPNMB mRNA levels of non-neoplastic renal tissues of BHD patients were as low as those of sporadic RCCs (data not shown).
Affiliation: Department of Molecular Pathology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.