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Loss of UCHL1 rescues the defects related to Parkinson’s disease by suppressing glycolysis

Su Jin Ham, D. S. Lee, Wen Jun Xu, Eunjoo Cho, Sekyu Choi, Soohong Min, Sunghyouk Park, Jongkyeong Chung

2021Science Advances61 citationsDOIOpen Access PDF

Abstract

and mammalian cells. In UCHL1 knockout cells, cellular pyruvate production and ATP levels are diminished, and the activity of AMP-activated protein kinase (AMPK) is highly induced. Consequently, the activated AMPK promotes the mitophagy mediated by Unc-51-like kinase 1 (ULK1) and FUN14 domain-containing 1 (FUNDC1), which underlies the effects of UCHL1 deficiency in rescuing PD-related defects. Furthermore, we identify tripartite motif-containing 63 (TRIM63) as a previously unknown E3 ligase of PKM and demonstrate its antagonistic interaction with UCHL1 to regulate PD-related pathologies. These results suggest that UCHL1 is an integrative factor for connecting glycolysis and PD pathology.

Topics & Concepts

MitophagyGlycolysisParkinson's diseaseEnergy metabolismDiseaseKinasePyruvate kinaseCell biologyMedicineNeuroscienceBiologyMetabolismBiochemistryInternal medicineAutophagyApoptosisAutophagy in Disease and TherapyParkinson's Disease Mechanisms and TreatmentsMitochondrial Function and Pathology