Results 31 to 40 of about 62,588 (334)

Regulation of CLC-1 chloride channel biosynthesis by FKBP8 and Hsp90β. [PDF]

open access: yes, 2016
Mutations in human CLC-1 chloride channel are associated with the skeletal muscle disorder myotonia congenita. The disease-causing mutant A531V manifests enhanced proteasomal degradation of CLC-1.
Chen, Shu-Ching   +7 more
core   +1 more source

Neuroprotection targeting protein misfolding on chronic cerebral hypoperfusion in the context of metabolic syndrome [PDF]

open access: yes, 2018
Metabolic syndrome (MetS) is a cluster of risk factors that lead to microvascular dysfunction and chronic cerebral hypoperfusion (CCH). Long-standing reduction in oxygen and energy supply leads to brain hypoxia and protein misfolding, thereby linking CCH
Capani, Francisco   +6 more
core   +1 more source

Dysregulated proteostasis network in neuronal diseases

open access: yesFrontiers in Cell and Developmental Biology, 2023
Long-term maintenance of synaptic connections is important for brain function, which depends on varying proteostatic regulations to govern the functional integrity of neuronal proteomes. Proteostasis supports an interconnection of pathways that regulates
Ching-San Tseng   +4 more
semanticscholar   +1 more source

Changes in Glutathione Redox Potential Are Linked to Aβ42-Induced Neurotoxicity

open access: yesCell Reports, 2018
Summary: Glutathione is the major low-molecular weight thiol of eukaryotic cells. It is central to one of the two major NADPH-dependent reducing systems and is likely to play a role in combating oxidative stress, a process suggested to play a key role in
Zeenna A. Stapper, Thomas R. Jahn
doaj   +1 more source

Proteostasis and Ribostasis Impairment as Common Cell Death Mechanisms in Neurodegenerative Diseases

open access: yesJournal of Clinical Neurology, 2023
The cellular homeostasis of proteins (proteostasis) and RNA metabolism (ribostasis) are essential for maintaining both the structure and function of the brain.
S. Lim, Minyeop Nahm, Seung Hyun Kim
semanticscholar   +1 more source

A Comprehensive Enumeration of the Human Proteostasis Network. 2. Components of the Autophagy-Lysosome Pathway

open access: yesbioRxiv, 2023
The condition of having a healthy, functional proteome is known as protein homeostasis, or proteostasis. Establishing and maintaining proteostasis is the province of the proteostasis network, approximately 2,700 components that regulate protein synthesis,
S. Elsasser   +30 more
semanticscholar   +1 more source

Seed-induced acceleration of amyloid-β mediated neurotoxicity in vivo

open access: yesNature Communications, 2017
Seeding of amyloid beta from one brain region to another is thought to contribute to the progression of Alzheimer’s disease, although to date most studies have depended on inoculation of animals with exogenous amyloid. Here the authors describe a genetic
Ramona F. Sowade, Thomas R. Jahn
doaj   +1 more source

The transcription factor ATF5: role in cellular differentiation, stress responses, and cancer. [PDF]

open access: yes, 2017
Activating transcription factor 5 (ATF5) is a cellular prosurvival transcription factor within the basic leucine zipper (bZip) family that is involved in cellular differentiation and promotes cellular adaptation to stress.
Angelastro, James M, Sears, Thomas K
core   +2 more sources

A mutational atlas for Parkin proteostasis

open access: yesbioRxiv, 2023
The delicate balance of protein homeostasis can be disturbed by mutations that affect folding and stability of the encoded protein. More than half of disease-causing missense variants are thought to lead to protein degradation, but determining which and ...
Lene Clausen   +12 more
semanticscholar   +1 more source

Biosensors for Studying Neuronal Proteostasis [PDF]

open access: yesFrontiers in Molecular Neuroscience, 2022
Cellular health depends on the integrity and functionality of the proteome. Each cell is equipped with a protein quality control machinery that maintains protein homeostasis (proteostasis) by helping proteins adopt and keep their native structure, and ensuring the degradation of damaged proteins.
openaire   +4 more sources

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