Figure 1 | Scientific Reports

Figure 1

From: Lymphoblastoid cell lines from Diamond Blackfan anaemia patients exhibit a full ribosomal stress phenotype that is rescued by gene therapy

Figure 1The alternative text for this image may have been generated using AI.

Characterisation of the pathological phenotype of LCLs from DBA patients. (A) Proliferation curve obtained by cell count. Growth rate in DBA LCLs was significantly lower than in controls after three days (p = 0.0043). Blue: controls, red: DBA patients. (B) Representative cell cycles of control and DBA cells; cells were fixed, stained with propidium iodide and subjected to flow cytometry. M1: subG1; M2: G0/G1; M3: S/G2/M. (C) Box plots reporting the mean percentages of cells in subG1 and S/G2/M phases. DBA patients show a higher proportion of cells in subG1 and a lower proportion of cells in S/G2/M phase, compared to controls. Ten control and 11 DBA LCLs were examined. Horizontal line represents median. **p value ≤ 0.01, ***p value ≤ 0.001. (D) General protein synthesis is decreased in DBA cells, as assessed by incorporation of [35S]methionine/cysteine. Horizontal line represents median. *p value ≤ 0.05. (E) Western blot shows an increase in the levels of p53, p21, MDM2 and phosphoAMPK (Thr172), whereas the level of total AMPK is unchanged. (F) Densitometric analysis on western blot data from three separate experiments. Horizontal line represents median. (G) Expression of p21 transcript, measured by qRT-PCR, is increased in DBA patients. Horizontal line represents median.

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