Cellular proteostasis in neurodegeneration

A Kurtishi, B Rosen, KS Patil, GW Alves… - Molecular neurobiology, 2019 - Springer
The term proteostasis reflects the fine-tuned balance of cellular protein levels, mediated
through a vast network of biochemical pathways. This requires the regulated control of …

Ubiquitin pathways in neurodegenerative disease

G Atkin, H Paulson - Frontiers in molecular neuroscience, 2014 - frontiersin.org
Control of proper protein synthesis, function, and turnover is essential for the health of all
cells. In neurons these demands take on the additional importance of supporting and …

The ubiquitin-proteasome system in neurodegeneration

C McKinnon, SJ Tabrizi - Antioxidants & redox signaling, 2014 - liebertpub.com
Significance: Impairment of the ubiquitin-proteasome system (UPS) has been implicated in
the pathogenesis of a wide variety of neurodegenerative disorders, including Alzheimer's …

Molecular chaperones' potential against defective proteostasis of amyotrophic lateral sclerosis

S Kinger, AR Dubey, P Kumar, YA Jagtap… - Cells, 2023 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a neuronal degenerative condition identified via a
build-up of mutant aberrantly folded proteins. The native folding of polypeptides is mediated …

E3 ubiquitin ligases neurobiological mechanisms: development to degeneration

A Upadhyay, V Joshi, A Amanullah, R Mishra… - Frontiers in molecular …, 2017 - frontiersin.org
Cells regularly synthesize new proteins to replace old or damaged proteins. Deposition of
various aberrant proteins in specific brain regions leads to neurodegeneration and aging …

SIRT1 overexpression ameliorates a mouse model of SOD1-linked amyotrophic lateral sclerosis via HSF1/HSP70i chaperone system

S Watanabe, N Ageta-Ishihara, S Nagatsu, K Takao… - Molecular brain, 2014 - Springer
Background Dominant mutations in superoxide dismutase 1 (SOD1) cause degeneration of
motor neurons in a subset of inherited amyotrophic lateral sclerosis (ALS). The pathogenetic …

From UBE3A to Angelman syndrome: a substrate perspective

GL Sell, SS Margolis - Frontiers in neuroscience, 2015 - frontiersin.org
Angelman syndrome (AS) is a debilitating neurodevelopmental disorder that is
characterized by motor dysfunction, intellectual disability, speech impairment, seizures and …

Azadiradione up-regulates the expression of parvalbumin and BDNF via Ube3a

S Jana, B Giri, S Das, A Manna, SC Mandal, NR Jana - Gene, 2024 - Elsevier
Azadiradione is a small bioactive limonoid found in the seed of Azadirachta Indica, an Indian
medicinal plant commonly known as Neem. Recently, it has been shown to ameliorate the …

Ubiquitin biology in neurodegenerative disorders: From impairment to therapeutic strategies

D Kumar, RK Ambasta, P Kumar - Ageing Research Reviews, 2020 - Elsevier
The abnormal accumulation of neurotoxic proteins is the typical hallmark of various age-
related neurodegenerative disorders (NDDs), including Alzheimer's disease, Parkinson's …

Insights into the ubiquitin-proteasome system of human embryonic stem cells

I Saez, S Koyuncu, R Gutierrez-Garcia, C Dieterich… - Scientific reports, 2018 - nature.com
Human embryonic stem cells (hESCs) exhibit high levels of proteasome activity, an intrinsic
characteristic required for their self-renewal, pluripotency and differentiation. However, the …