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This work was supported in part by a research grant from Core Research for Evolutional Science and Technology (CREST) of Japan Science and Technology Cooperation (J.S.T.), Grant-in-Aid for Scientific Research on Priority Areas from the Ministry of Education, Science, Sports and Culture, and a grant of R and D Projects in Cooperation with Academic Institutions from New Energy and Industrial Technology Development Organization (NEDO)

Maralixibat was not beneficial in palliating pruritus in the severe phenotype of our patient

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The downstream target of Nrf2, NQO1, has also been implicated in protection against diabetes and metabolic syndrome