Add to ORKGDefects in the mitochondrial respiratory chain (RC) underlie a spectrum of human conditions, ranging from devastating inborn errors of metabolism to aging. We performed a genome-wide Cas9-mediated screen to identify factors that are protective during RC inhibition. Our results highlight the hypoxia response, an endogenous program evolved to adapt to limited oxygen availability. Genetic or small-molecule activation of the hypoxia response is protective against mitochondrial toxicity in cultured cells and zebrafish models. Chronic hypoxia leads to a marked improvement in survival, body weight, body temperature, behavior, neuropathology, and disease biomarkers in a genetic mouse model of Leigh syndrome, the most common pediatric manifestation ...
Background: In most eukaryotic cells, the mitochondrial DNA (mtDNA) is transmitted uniparentally and...
Mitochondria are the major source of ATP that is synthesized by the respiratory chain through the pr...
Mitochondria play a pivotal role in controlling cellular homeostasis. The mitochondrial respiratory ...
Leigh syndrome is a devastating mitochondrial disease for which there are no proven therapies. We pr...
Dysfunctions of the mitochondrial electron transport chain cause severe, currently untreatable, dise...
Background: F1F0-ATP synthase (F1F0-ATPase) plays important roles in regulating mitochondrial functi...
In addition to ATP generation, mitochondria are essential in various cellular processes ranging from...
Thesis (Ph.D.)--University of Rochester. School of Medicine & Dentistry. Dept. of Pathology, 2017.Mi...
Mitochondria are network-like organelles present in most mammalian cells. They contain the respirato...
Mitochondria are essential for cell survival and health, utilizing about 90% of the oxygen we breath...
Radiation induced brain injury (RBI) is the most serious adverse reaction in cranial radiotherapy, w...
Mitochondria are essential for cell survival and health, utilizing about 90% of the oxygen we breath...
Oxidative stress is a distinctive sign in several genetic disorders characterized by cancer predispo...
Background: Many age-associated disorders (including diabetes, cancer, and neurodegenerative disease...
Mutations of mitochondrial DNA cause a variety of clinical syndromes. It is unclear whether impaired...
Background: In most eukaryotic cells, the mitochondrial DNA (mtDNA) is transmitted uniparentally and...
Mitochondria are the major source of ATP that is synthesized by the respiratory chain through the pr...
Mitochondria play a pivotal role in controlling cellular homeostasis. The mitochondrial respiratory ...
Leigh syndrome is a devastating mitochondrial disease for which there are no proven therapies. We pr...
Dysfunctions of the mitochondrial electron transport chain cause severe, currently untreatable, dise...
Background: F1F0-ATP synthase (F1F0-ATPase) plays important roles in regulating mitochondrial functi...
In addition to ATP generation, mitochondria are essential in various cellular processes ranging from...
Thesis (Ph.D.)--University of Rochester. School of Medicine & Dentistry. Dept. of Pathology, 2017.Mi...
Mitochondria are network-like organelles present in most mammalian cells. They contain the respirato...
Mitochondria are essential for cell survival and health, utilizing about 90% of the oxygen we breath...
Radiation induced brain injury (RBI) is the most serious adverse reaction in cranial radiotherapy, w...
Mitochondria are essential for cell survival and health, utilizing about 90% of the oxygen we breath...
Oxidative stress is a distinctive sign in several genetic disorders characterized by cancer predispo...
Background: Many age-associated disorders (including diabetes, cancer, and neurodegenerative disease...
Mutations of mitochondrial DNA cause a variety of clinical syndromes. It is unclear whether impaired...
Background: In most eukaryotic cells, the mitochondrial DNA (mtDNA) is transmitted uniparentally and...
Mitochondria are the major source of ATP that is synthesized by the respiratory chain through the pr...
Mitochondria play a pivotal role in controlling cellular homeostasis. The mitochondrial respiratory ...