The endoplasmic reticulum (ER) chaperone BiP is a master regulator of ER functions: Getting by with a little help from ERdj friends
The endoplasmic reticulum (ER) represents the entry point into the secretory pathway where
nascent proteins encounter a specialized environment for their folding and maturation …
nascent proteins encounter a specialized environment for their folding and maturation …
Regulation of calcium homeostasis and flux between the endoplasmic reticulum and the cytosol
L Daverkausen-Fischer, F Pröls - Journal of Biological Chemistry, 2022 - ASBMB
The concentration of Ca 2+ in the endoplasmic reticulum (ER) is critically important for
maintaining its oxidizing environment as well as for maintaining luminal ATP levels required …
maintaining its oxidizing environment as well as for maintaining luminal ATP levels required …
Glucocerebrosidase is imported into mitochondria and preserves complex I integrity and energy metabolism
Mutations in GBA1, the gene encoding the lysosomal enzyme β-glucocerebrosidase
(GCase), which cause Gaucher's disease, are the most frequent genetic risk factor for …
(GCase), which cause Gaucher's disease, are the most frequent genetic risk factor for …
Rescue of α-synuclein aggregation in Parkinson's patient neurons by synergistic enhancement of ER proteostasis and protein trafficking
Neurodegenerative disorders are characterized by a collapse in proteostasis, as shown by
the accumulation of insoluble protein aggregates in the brain. Proteostasis involves a …
the accumulation of insoluble protein aggregates in the brain. Proteostasis involves a …
How do J-proteins get Hsp70 to do so many different things?
EA Craig, J Marszalek - Trends in biochemical sciences, 2017 - cell.com
Hsp70 chaperone machineries have pivotal roles in an array of fundamental biological
processes through their facilitation of protein folding, disaggregation, and remodeling. The …
processes through their facilitation of protein folding, disaggregation, and remodeling. The …
[HTML][HTML] Glucocerebrosidase-associated Parkinson disease: Pathogenic mechanisms and potential drug treatments
ME Gegg, E Menozzi, AHV Schapira - Neurobiology of Disease, 2022 - Elsevier
Dysfunction of the endolysosomal system is implicated in the pathogenesis of both sporadic
and familial Parkinson disease (PD). Variants in genes encoding lysosomal proteins have …
and familial Parkinson disease (PD). Variants in genes encoding lysosomal proteins have …
Close and allosteric opening of the polypeptide-binding site in a human Hsp70 chaperone BiP
Binding immunoglobulin protein (BiP), an essential and ubiquitous Hsp70 chaperone in the
ER, plays a key role in protein folding and quality control. BiP contains two functional …
ER, plays a key role in protein folding and quality control. BiP contains two functional …
Converging roles of ion channels, calcium, metabolic stress, and activity pattern of Substantia nigra dopaminergic neurons in health and Parkinson's disease
J Duda, C Pötschke, B Liss - Journal of neurochemistry, 2016 - Wiley Online Library
Dopamine‐releasing neurons within the Substantia nigra (SN DA) are particularly
vulnerable to degeneration compared to other dopaminergic neurons. The age‐dependent …
vulnerable to degeneration compared to other dopaminergic neurons. The age‐dependent …
Lysosome and Inflammatory Defects in GBA1‐Mutant Astrocytes Are Normalized by LRRK2 Inhibition
A Sanyal, MP DeAndrade, HS Novis, S Lin… - Movement …, 2020 - Wiley Online Library
Background Autosomal recessive mutations in the glucocerebrosidase gene, Beta‐
glucocerebrosidase 1 (GBA1), cause the lysosomal storage disorder Gaucher's disease …
glucocerebrosidase 1 (GBA1), cause the lysosomal storage disorder Gaucher's disease …
Protein quality control in the endoplasmic reticulum
BM Adams, ME Oster, DN Hebert - The protein journal, 2019 - Springer
The site of protein folding and maturation for the majority of proteins that are secreted,
localized to the plasma membrane or targeted to endomembrane compartments is the …
localized to the plasma membrane or targeted to endomembrane compartments is the …