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. 2021 Jun 28;10(7):1616. doi: 10.3390/cells10071616

Figure 1.

Figure 1

Deficient RNA Pol I transcription and altered rRNA processing in CS cells. (A) Quantitative PCR analysis of 47S, 28S/ETS, 5.8S/ITS2, and mature 18S rRNA. Used cell lines were wt (1306 fibroblasts), CSArec (CS3BE transfected with HA-CSA), CSAmut (CS3BE), CSBrec (CS1AN, transfected with HA-CSB), CSBmut (CS1AN), and UVsKO (UV-sensitive cells). A scheme of the primer positions is provided above. (B) Schema of the rRNA processing pathway in human cells, adapted from Mullineux and Lafontaine [25]. The ITS1 probe is identified in purple; the ITS2 probe is in orange. (C) Northern blot image of control cells, CS mutated cells, and reconstituted cells. (D) RAMP analysis of the Northern blots reveals a relative accumulation of processing intermediates in CS patient cells. Quantification and statistics are provided in the Supplementary Material (Figure S1). Asteriks (*) in the figures represent ρ values (* p < 0.05, ** p < 0.01, *** p < 0.001, **** p < 0.0001).