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Integrated metabolic and proteostatic profiling reveals remodeling of proteolytic pathways associated with redox-bioenergetic dysfunction in a PAH <sup>enu2</sup> mouse model of phenylketonuria

2026-07-09

Abstract excerpt

Phenylketonuria (PKU) is a genetic metabolic disorder caused by the lack of functional phenylalanine hydroxylase (PAH). Elevated levels of phenylalanine (Phe) are known to be neurotoxic; however, the molecular mechanisms underlying Phe’s effects remain elusive. This study investigates the impact of PKU on proteostasis, redox balance, and metabolism in BTBR PAH enu2 mice, a severe disease animal model. Combined p...

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Literature Corpus work
e734af77-c5e3-55e3-a7b2-e1161ab38fdd
DOI
10.64898/2026.07.08.736353
Open publication

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Integrated metabolic and proteostatic profiling reveals remodeling of proteolytic pathways associated with redox-bioenergetic dysfunction in a PAH <sup>enu2</sup> mouse model of phenylketonuriaDOI 10.64898/2026.07.08.736353
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