Results 91 to 100 of about 543,462 (281)

Autophagosome marker, LC3, is released extracellularly via several distinct pathways

FEBS Open Bio, EarlyView.
This study establishes a novel HiBiT‐tagging system for ultrasensitive detection of LC3, revealing multiple pathways for its extracellular secretion. It demonstrates that LC3 is released via both autophagy‐dependent and ‐independent mechanisms, including a novel route for nonlipidated LC3‐I.
Koki Saito, Masashi Arakawa, Koki Maeda, Eiji Morita   +3 more
wiley   +1 more source

SIRT4 positively regulates autophagy via ULK1, but independently of HDAC6 and OPA1

FEBS Open Bio, EarlyView.
Cells expressing SIRT4 (H161Y), a catalytically inactive mutant of the sirtuin SIRT4, fail to upregulate LC3B‐II and exhibit a reduced autophagic flux under stress conditions. Interestingly, SIRT4(H161Y) promotes phosphorylation of ULK1 at S638 and S758 that are associated with inhibition of autophagy initiation.
Isabell Lehmkuhl, Khawar Amin, Lydia Gabriel, Nils Hampel, Afshin Iram, Julia Hesse, Constanze Wiek, Jasmin Thuy Vy Nguyen, Helmut Hanenberg, Jürgen Scheller, M. Reza Ahmadian, Björn Stork, Doreen M. Floss, Roland P. Piekorz   +13 more
wiley   +1 more source

A unified mechanism for mitochondrial damage sensing in PINK1-Parkin–mediated mitophagy

The EMBO Journal
Damaged mitochondria can be cleared from the cell by mitophagy, using a pathway formed by the recessive Parkinson’s disease genes PINK1 and Parkin. Whether the pathway senses diverse forms of mitochondrial damage via a common mechanism, however, remains ...
Julia A Thayer, Jennifer D Petersen, Xiaoping Huang, Luiza M Gruel Budet, James Hawrot, Daniel M Ramos, Shiori Sekine, Yan Li, Michael E Ward, Derek P Narendra   +9 more
doaj   +1 more source

A case with short stature and proteinuria: atypical presentation of a family with m.3243A>G mutation

The Turkish Journal of Pediatrics
Background. The mitochondrial DNA (mtDNA) m.3243A>G mutation is one of the most common pathogenic mtDNA variants. The phenotypes associated with this mutation range from asymptomatic induviduals to well-defined clinical syndromes, or non-syndromic ...
Gönül Büyükyılmaz, Mihriban İnözü, Büşranur Çavdarlı   +2 more
doaj   +1 more source

Metformin promotes mitochondrial integrity through AMPK‐signaling in Leber's hereditary optic neuropathy

FEBS Open Bio, EarlyView.
Metformin mediates mitochondrial quality control in Leber's hereditary optic neuropathy (LHON) fibroblasts carrying mtDNA mutations. At therapeutic levels, metformin activates AMPK signaling to restore mitochondrial dynamics by promoting fusion and restraining fission, while preserving mitochondrial mass, enhancing autophagy/mitophagy and biogenesis ...
Chatnapa Panusatid, Rapasviranda Soiyangsuk, Maneeluck Tanadjindarat, Chayanon Peerapittayamongkol   +3 more
wiley   +1 more source

Zagociguat prevented stressor-induced neuromuscular dysfunction, improved mitochondrial physiology, and increased exercise capacity in diverse mitochondrial respiratory chain disease zebrafish models

Frontiers in Pharmacology
BackgroundZagociguat (zag) is a CNS-penetrant, soluble guanylate cyclase (sGC) stimulator that has been evaluated in phase 2a, with phase 2b ongoing, clinical studies of primary mitochondrial disease (PMD) subjects with mitochondrial encephalomyopathy ...
Leonard Burg, Heeyong Yoon, Min Peng, Peter Germano, Emily Reesey Gretzmacher, Rui Xiao, Vernon E. Anderson, Eiko Nakamaru-Ogiso, Eiko Nakamaru-Ogiso, Marni J. Falk, Marni J. Falk   +10 more
doaj   +1 more source

P1‐121: Generation and characterization of neuron‐specific mitochondrial targeted DsRed2 transgenic mouse model to image synapse modification in Alzheimer's disease [PDF]

, 2008
Yi Wang, Lee J. Martin
openalex   +1 more source

Oxidized mitochondrial DNA released after inflammasome activation is a disease biomarker for myelodysplastic syndromes [PDF]

, 2021
Grace Ward, Kathy L. McGraw, Farnoosh Abbas‐Aghababazadeh, Benjamin S. Meyer, Amy F. McLemore, Nicole D. Vincelette, Nghi B. Lam, Amy L. Aldrich, Najla H. Al Ali, Eric Padron, Javier Pinilla‐Ibarz, Erico Masala, Valeria Santini, Olivier Kosmider, Michaëla Fontenay, Pierre Fenaux, Joseph Johnson, Brooke L. Fridley, Alan F. List   +18 more
openalex   +1 more source

Evaluation of in vitro toxicity of common phytochemicals included in weight loss supplements using 1H NMR spectroscopy

FEBS Open Bio, EarlyView.
We investigated the toxicity of 12 active compounds commonly found in herbal weight loss supplements (WLS) using human liver and colon cell models. Epigallocatechin‐3‐gallate was the only compound showing significant toxicity. Metabolic profiling revealed protein degradation, disrupted energy and lipid metabolism suggesting that the inclusion of EGCG ...
Emily C. Davies, Garth L. Maker, Ian F. Musgrave, Samantha Lodge   +3 more
wiley   +1 more source

Mitochondrial Diseases

In this review the author describes the current knowledge about mitochondrial diseases (mitochondrial myopathies, mitochondrial encephalomyopathies and mitochondrial cardiomyopathies) in both clinical and experimental medicine. This description concerns historical development of mitochondrial diseases, biochemical alterations and genetic disturbances ...
openaire   +4 more sources