Defective Mitochondrial Cardiolipin Remodeling Dampens HIF-1α Expression in Hypoxia
Sign Up to like & getrecommendations! Published in 2018 at "Cell Reports"
DOI: 10.1016/j.celrep.2018.09.057
Abstract: Summary Mitochondria fulfill vital metabolic functions and act as crucial cellular signaling hubs, integrating their metabolic status into the cellular context. Here, we show that defective cardiolipin remodeling, upon loss of the cardiolipin acyl transferase… read more here.
Keywords: cardiolipin remodeling; remodeling dampens; cardiolipin; mitochondrial cardiolipin ... See more keywords
Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome
Sign Up to like & getrecommendations! Published in 2017 at "Human Molecular Genetics"
DOI: 10.1093/hmg/ddx314
Abstract: Abstract Defects in nuclear-encoded proteins of the mitochondrial translation machinery cause early-onset and tissue-specific deficiency of one or more OXPHOS complexes. Here, we report a 7-year-old Italian boy with childhood-onset rapidly progressive encephalomyopathy and stroke-like… read more here.
Keywords: rrna; causes melas; mrm2 causes; melas like ... See more keywords
Molecular pathways in mitochondrial disorders due to a defective mitochondrial protein synthesis
Sign Up to like & getrecommendations! Published in 2024 at "Frontiers in Cell and Developmental Biology"
DOI: 10.3389/fcell.2024.1410245
Abstract: Mitochondria play a central role in cellular metabolism producing the necessary ATP through oxidative phosphorylation. As a remnant of their prokaryotic past, mitochondria contain their own genome, which encodes 13 subunits of the oxidative phosphorylation… read more here.
Keywords: mitochondrial protein; defective mitochondrial; protein; translation ... See more keywords