Cholesterol ensures ciliary polycystin-2 localization to prevent polycystic kidney disease
Peroxisome-mediated cholesterol trafficking is essential for the ciliary localization of the polycystin complex to prevent the occurrence of polycystic kidney.
Takeshi Itabashi +13 more
doaj +1 more source
Mitochondrial membrane remodeling in stress adaptation: Lipid control of organelle quality
Abstract Mitochondria respond to proteotoxic stress through the mitochondrial unfolded protein response, traditionally viewed as a transcriptional program that restores proteostasis by inducing chaperones and proteases. Emerging evidence indicates that mitochondrial membrane remodeling constitutes an additional adaptive component of this response ...
Lena J. Reichert +2 more
wiley +1 more source
Peroxisomal protein translocation [PDF]
Peroxisomes perform a wide variety of metabolic processes in eukaryotic organisms. Mutations that affect peroxisome function or formation have profound phenotypic consequences, the latter demonstrated by peroxisome biogenesis disorders which are often ...
Saffian, Delia +2 more
core +1 more source
Peroxisomal import stress activates integrated stress response and inhibits ribosome biogenesis [PDF]
Impaired organelle-specific protein import triggers a variety of cellular stress responses, including adaptive pathways to balance protein homeostasis. Most of the previous studies focus on the cellular stress response triggered by misfolded proteins or ...
Kim, Jinoh +7 more
core +3 more sources
The estrogen deficiency in the hippocampus influences cognitive functions and control of food intake. Hippocampi of female rats, either control or ovariectomized, with or without estrogen replacement, were submitted to LC–MS/MS and analysis of pathway‐based functional roles of proteins affected by estrogen deficiency and replacement.
Amanda Paula Pedroso +8 more
wiley +1 more source
Fibroblast growth factor receptor 1 (FGFR1) is co‐translationally integrated into endoplasmic reticulum (ER) membranes and undergoes N‐glycosylation at several positions. This process is essential for FGFR1 progression through the secretory pathway.
Paulina Działek +5 more
wiley +1 more source
Peroxisomal Proteostasis Involves a Lon Family Protein That Functions as Protease and Chaperone [PDF]
Proteins are subject to continuous quality control for optimal proteostasis. The knowledge of peroxisome quality control systems is still in its infancy. Here we show that peroxisomes contain a member of the Lon family of proteases (Pln).
Kikhney, Alexey +24 more
core +3 more sources
Airway Involvement in Conradi–Hünermann–Happle Syndrome: A Novel Clinical Manifestation
We report the first documented case of airway involvement in Conradi–Hünermann–Happle syndrome (CDPX2), an X‐linked dominant form of chondrodysplasia punctata caused by pathogenic variants in EBP. A 2‐month‐old female with genetically confirmed CDPX2 developed severe subglottic stenosis and persistent respiratory distress requiring CPAP; cross ...
Enrique G. Villarreal +3 more
wiley +1 more source
The TRIM37 Gene Encodes a Peroxisomal RING-B-Box-Coiled-Coil Protein: Classification of Mulibrey Nanism as a New Peroxisomal Disorder [PDF]
Mulibrey nanism is a rare growth disorder of prenatal onset caused by mutations in the TRIM37 gene, which encodes a RING-B-box-coiled-coil protein. The pathogenetic mechanisms of mulibrey nanism are unknown. We have used transiently transfected cells and
Ulmanen, Ismo +4 more
core +1 more source
Metabolic Kinases as Regulators of Inter‐Organelle Communication in Aging and Age‐Related Diseases
Metabolic kinases integrate cellular energy, nutrient, and stress signals to regulate inter‐organelle coordination during aging. In young or healthy cells, coordinated communication among mitochondria, the endoplasmic reticulum, lysosomes, peroxisomes, and the Golgi apparatus supports calcium handling, lipid metabolism, mitochondrial dynamics ...
Md Riad Chowdhury +2 more
wiley +1 more source

