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A role for cytochrome c and cytochrome c peroxidase in electron shuttling from Erv1
TLDR
It is proposed that Erv1 utilizes diverse pathways for electron shuttling in the IMS by coupling these pathways, cytochrome c and Ccp1 function efficiently as ErV1‐dependent electron acceptors. Expand
Transgenic Mouse Model of Tau Pathology in Astrocytes Leading to Nervous System Degeneration
TLDR
The transgenic Tg mice recapitulate key features of astrocytic pathology observed in human tauopathies and demonstrate functional consequences of this pathology including neuron degeneration in the absence of neuronal tau inclusions. Expand
Expression of the small heat-shock protein alphaB-crystallin in tauopathies with glial pathology.
TLDR
Increased expression of the small HSP alphaB-crystallin in glial tau inclusions may represent a response by glia to the accumulation of misfolded or aggregated tau protein that is linked to the pathogenesis of the glial pathology and distinct from mechanisms underlying neuronal tau pathology in neurodegenerative disease. Expand
Impaired Glutamate Transport in a Mouse Model of Tau Pathology in Astrocytes
TLDR
A transgenic mouse model of tau pathology in astrocytes is generated by expressing the human tau protein under the control of the glial fibrillary acidic protein (GFAP) promoter and it is demonstrated that compromised motor function and deficits in neuromuscular strength are observed that correlates with reduced expression of glutamate transporter-1 and occurs concurrent with tau inclusion pathology. Expand
Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins.
TLDR
It is demonstrated that Mia40, Erv1, and oxygen are the minimal machinery for Tim13 oxidation. Expand
A small molecule inhibitor of redox-regulated protein translocation into mitochondria.
TLDR
MitoBloCK-6 induced apoptosis via cytochrome c release in human embryonic stem cells (hESCs) but not in differentiated cells, suggesting an important role for ALR in hESC homeostasis and an unexpected role for Erv1 in the carrier import pathway. Expand
Substrate specificity of the TIM22 mitochondrial import pathway revealed with small molecule inhibitor of protein translocation
TLDR
A chemical-genetic approach to identify small molecule agonists that caused lethality to a tim10-1 yeast mutant at the permissive temperature validates the generation of new probes to facilitate mechanistic studies in yeast and mammalian mitochondria and confirms MitoBloCK-1 can be used to understand mammalian mitochondrial import and dysfunction linked to inherited human disease. Expand
Role of Twin Cys-Xaa9-Cys Motif Cysteines in Mitochondrial Import of the Cytochrome c Oxidase Biogenesis Factor Cmc1*
TLDR
The results suggest that the in vitro and in organello oxidative folding of Cmc1, a twin CX9C-containing substrate, which contains an unpaired cysteine, facilitates efficient formation of multiple disulfides and prevents the formation of non-native disulfide bonds. Expand
Proteasome Inhibition Stabilizes Tau Inclusions in Oligodendroglial Cells that Occur after Treatment with Okadaic Acid
TLDR
It is shown by immunohistochemistry that tau-positive oligodendroglial inclusion bodies also contain the small heat-shock protein (HSP) αB-crystallin but not HSP70, and only hyperphosporylation of tau followed by proteasome inhibition leads to stable fibrillary deposits of tAU similar to those observed in neurodegenerative diseases. Expand
In vivo imaging of mitochondrial membrane potential in non-small-cell lung cancer
TLDR
A positron emission tomography imaging tracer is developed to image mitochondrial function in vivo, and application of this tracer to a mouse model of lung cancer identifies distinct functional mitochondrial heterogeneity within subtypes of lung tumours. Expand
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