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. 2014 Aug 7;4(3):640–654. doi: 10.3390/metabo4030640

Figure 1.

Figure 1

Hereditary leiomyomatosis and renal cell cancer (HLRCC) tumours and tumour-derived cell lines express high levels of 2SC. (A) Immunoblotting confirms the presence of fumarate hydratase (FH) in the control human renal proximal tubular epithelial (RPTC) cell line (CTL), the presence of the mutant FH allele in the UOK262 (UOK) HLRCC-derived cell line as previously described and absence of FH protein in the NCC-FH-1 (NCC) HLRCC-derived cell line. β-actin levels were used as a loading control for each cell line. (B) Immunoblotting for 2SC clearly shows an absence of 2SC expression in the control cell line but high expression of 2SC in the FH-mutant cell lines (UOK and NCC). (C) Positive 2SC immunoreactivity (indicated by brown staining) is evident in the tumour cells of an FH mutant type 2 PRCC (HLRCC PRCC) but absent in the stromal tissue (blue staining).