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Vascular relaxation in an endothelial-dependent manner was dysfunctional, including the maximal relaxation despite no change in smooth muscle-dependent vasorelaxation (Fig

In the nonprocessing state, the NUB1FAT10Eos complex showed only weak, unresolvable density that likely originates from an intrinsic flexibility within the NUB1 complex itself and from its binding to Rpn1, which is the most mobile subunit of the 19S regulatory particle (Fig

MMP, matrix metalloprotein

Clinical indications of cholestasis (discoloured faeces, dark urine) frequently occur in the first few months of infancy (with a tendency to appear earlier than PFIC1), with recurring or persistent jaundice, low GT, elevated BAs and FP, hepatomegaly, and severe pruritus