title: The COP9 signalosome coerces autophagy and the ubiquitin-proteasome system to police the heart.
abstract: We demonstrated for the first time that the COP9 signalosome (COPS) controls the degradation of a surrogate and a bona fide misfolded protein in the cytosol of cardiomyocytes likely via supporting ubiquitination by CUL/cullin-RING ligases, and that Cops8 hypomorphism exacerbates cardiac proteinopathy in mice, in which autophagic impairment appears to be in play. It will be extremely imprtant to investigate cardiac ablation of another Cops gene to decipher whether COPS8 deficiency phenotypes are attributable to the COPS or unique to COPS8.
authors.affiliation: a State Key Laboratory of Respiratory Disease and Department of Pathophysiology , Guangzhou Medical University , Guangzhou , Guangdong , China.
authors.forename: Jinbao
authors.initials: J
authors.lastname: Liu
authors.affiliation: b Vascular Biology Center and Department of Pharmacology and Toxicology , Medical College of Georgia, Georgia Regents University , Augusta , GA , USA.
authors.forename: Huabo
authors.initials: H
authors.lastname: Su
authors.affiliation: a State Key Laboratory of Respiratory Disease and Department of Pathophysiology , Guangzhou Medical University , Guangzhou , Guangdong , China.|c Division of Basic Biomedical Sciences, Sanford School of Medicine of the University of South Dakota , Vermillion , SD , USA.
authors.forename: Xuejun
authors.initials: X
authors.lastname: Wang
mesh_terms: D000818:Animals; D001343:Autophagy; D000075686:COP9 Signalosome Complex; D044842:Cullin Proteins / Q000378:metabolism; D006321:Heart / Q000502:physiology*; D051379:Mice; D046912:Multiprotein Complexes / Q000378:metabolism*; D010447:Peptide Hydrolases / Q000378:metabolism*; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism*; D025801:Ubiquitin / Q000378:metabolism*
chemical_list: D044842:Cullin Proteins; D046912:Multiprotein Complexes; D025801:Ubiquitin; D010447:Peptide Hydrolases; D000075686:COP9 Signalosome Complex; D046988:Proteasome Endopeptidase Complex
keywords: COP9 signalosome; autophagy; cullin-RING ligase; desmin-related cardiomyopathy; protein quality control