Disruption of axonal transport in neurodegeneration
SH Berth, TE Lloyd - The Journal of Clinical Investigation, 2023 - Am Soc Clin Investig
Neurons are markedly compartmentalized, which makes them reliant on axonal transport to
maintain their health. Axonal transport is important for anterograde delivery of newly …
maintain their health. Axonal transport is important for anterograde delivery of newly …
Genetics of amyotrophic lateral sclerosis: seeking therapeutic targets in the era of gene therapy
N Suzuki, A Nishiyama, H Warita, M Aoki - Journal of human genetics, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is an intractable disease that causes respiratory failure
leading to mortality. The main locus of ALS is motor neurons. The success of antisense …
leading to mortality. The main locus of ALS is motor neurons. The success of antisense …
Deficient endoplasmic reticulum-mitochondrial phosphatidylserine transfer causes liver disease
MI Hernández-Alvarez, D Sebastián, S Vives… - Cell, 2019 - cell.com
Non-alcoholic fatty liver is the most common liver disease worldwide. Here, we show that the
mitochondrial protein mitofusin 2 (Mfn2) protects against liver disease. Reduced Mfn2 …
mitochondrial protein mitofusin 2 (Mfn2) protects against liver disease. Reduced Mfn2 …
Small molecule agonist of mitochondrial fusion repairs mitochondrial dysfunction
Y Guo, H Zhang, C Yan, B Shen, Y Zhang… - Nature chemical …, 2023 - nature.com
Membrane dynamics are important to the integrity and function of mitochondria. Defective
mitochondrial fusion underlies the pathogenesis of multiple diseases. The ability to target …
mitochondrial fusion underlies the pathogenesis of multiple diseases. The ability to target …
Mitochondrial function in development and disease
MP Rossmann, SM Dubois… - Disease models & …, 2021 - journals.biologists.com
Mitochondria are organelles with vital functions in almost all eukaryotic cells. Often
described as the cellular 'powerhouses' due to their essential role in aerobic oxidative …
described as the cellular 'powerhouses' due to their essential role in aerobic oxidative …
Axonal endoplasmic reticulum dynamics and its roles in neurodegeneration
The physical continuity of axons over long cellular distances poses challenges for their
maintenance. One organelle that faces this challenge is endoplasmic reticulum (ER); unlike …
maintenance. One organelle that faces this challenge is endoplasmic reticulum (ER); unlike …
Cellular senescence links mitochondria-ER contacts and aging
DV Ziegler, N Martin, D Bernard - Communications Biology, 2021 - nature.com
Membrane contact sites emerged in the last decade as key players in the integration,
regulation and transmission of many signals within cells, with critical impact in multiple …
regulation and transmission of many signals within cells, with critical impact in multiple …
TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration
Genetic mutations in TAR DNA-binding protein 43 (TDP-43) cause amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). Importantly, TDP-43 proteinopathy …
sclerosis (ALS) and frontotemporal dementia (FTD). Importantly, TDP-43 proteinopathy …
MFN2 mutations in Charcot–Marie–Tooth disease alter mitochondria-associated ER membrane function but do not impair bioenergetics
D Larrea, M Pera, A Gonnelli… - Human molecular …, 2019 - academic.oup.com
Abstract Charcot–Marie–Tooth disease (CMT) type 2A is a form of peripheral neuropathy,
due almost exclusively to dominant mutations in the nuclear gene encoding the …
due almost exclusively to dominant mutations in the nuclear gene encoding the …
Contribution of proteases to the hallmarks of aging and to age‐related neurodegeneration
Protein quality control ensures the degradation of damaged and misfolded proteins.
Derangement of proteostasis is a primary cause of aging and age‐associated diseases. The …
Derangement of proteostasis is a primary cause of aging and age‐associated diseases. The …