Whether or not RTEL1 travels with the pre-U2 RNP through the nuclear pore or just licenses it for transport remains to be determined; nevertheless, the large cytoplasmic accumulations of PHAX, the XPO1-RNP adaptor, seen both in the absence and upon expression of mutated forms of RTEL1 (as well as in RTEL1-HHS patients’ cells), provide strong evidence that RTEL1 is also exerting a crucial role in the cytoplasm. The gene discussed is RTEL1; the disease is hypotrichosis 1.