Results 101 to 110 of about 212,226 (348)

Large‐scale bidirectional arrayed genetic screens identify OXR1 and EMC4 as modifiers of αSynuclein aggregation

open access: yesFEBS Open Bio, EarlyView.
Activation of the mitochondrial protein OXR1 increases pSyn129 αSynuclein aggregation by lowering ATP levels and altering mitochondrial membrane potential, particularly in response to MSA‐derived fibrils. In contrast, ablation of the ER protein EMC4 enhances autophagic flux and lysosomal clearance, broadly reducing α‐synuclein aggregates.
Sandesh Neupane   +11 more
wiley   +1 more source

H+ transport is an integral function of the mitochondrial ADP/ATP carrier. [PDF]

open access: yes, 2019
The mitochondrial ADP/ATP carrier (AAC) is a major transport protein of the inner mitochondrial membrane. It exchanges mitochondrial ATP for cytosolic ADP and controls cellular production of ATP.
Angelin, Alessia   +12 more
core   +1 more source

Super‐Refractory Status Epilepticus (SRSE) in a Patient With Compound Heterozygous OPA1 Variants: Case Report and Literature Review

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Super‐Refractory Status Epilepticus (SRSE) is a rare, life‐threatening neurological emergency with unclear etiology in many cases. Mitochondrial dysfunction, often due to disease‐causing genetic variants, is increasingly recognized as a cause, with each gene producing distinct pathophysiological mechanisms.
Pouria Mohammadi   +2 more
wiley   +1 more source

TIM23-mediated insertion of transmembrane alpha-helices into the mitochondrial inner membrane [PDF]

open access: yes, 2011
While overall hydrophobicity is generally recognized as the main characteristic of transmembrane (TM) alpha-helices, the only membrane system for which there are detailed quantitative data on how different amino acids contribute to the overall efficiency
Björkholm, Patrik   +7 more
core  

Detailed Analysis of the Human Mitochondrial Contact Site Complex Indicate a Hierarchy of Subunits [PDF]

open access: yes, 2015
Mitochondrial inner membrane folds into cristae, which significantly increase its surface and are important for mitochondrial function. The stability of cristae depends on the mitochondrial contact site (MICOS) complex.
Dorsch, Eva   +4 more
core   +3 more sources

Evidence of Iron Accumulation in Cerebral Adrenoleukodystrophy: A Potential Novel Disease Mechanism

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT In this first application of Quantitative Susceptibility Mapping Source Separation to cerebral adrenoleukodystrophy, we uncovered alterations in iron and myelin within lesions and normal appearing white matter. As validation, we demonstrate abnormal iron accumulation in those same compartments within primary brain tissue.
Christina L. Nemeth   +8 more
wiley   +1 more source

Enhancing the Mitochondrial Uptake of Phosphonium Cations by Carboxylic Acid Incorporation

open access: yesFrontiers in Chemistry, 2020
There is considerable interest in developing drugs and probes targeted to mitochondria in order to understand and treat the many pathologies associated with mitochondrial dysfunction.
Laura Pala   +10 more
doaj   +1 more source

Structural analysis of a trimeric assembly of the mitochondrial dynamin-like GTPase Mgm1. [PDF]

open access: yes, 2020
The fusion of inner mitochondrial membranes requires dynamin-like GTPases, Mgm1 in yeast and OPA1 in mammals, but how they mediate membrane fusion is poorly understood.
Hu, Junjie   +13 more
core  

Ergosterol reduction impairs mitochondrial DNA maintenance in S. cerevisiae [PDF]

open access: yes, 2019
Sterols are essential lipids, involved in many biological processes. In Saccharomyces cerevisiae, the enzymes of the ergosterol biosynthetic pathway (Erg proteins) are localized in different cellular compartments.
Balliano, Gianni   +6 more
core   +2 more sources

Synthetic Nanobiology Actuated Lipometabolic Cell Factory for Autologous Tumor Immunotherapy

open access: yesAdvanced Functional Materials, EarlyView.
FA plays a crucial role in the interaction between tumor cells and the tumor microenvironment, especially for the immune response. A biocatalytic immunoenhancement strategy is developed to boost antitumor immunity by FA metabolic orientation to ceramide. Through the design of this delicate catalytic immunoenhancement strategy, the synthetic nanobiology
Shoujie Zhao   +8 more
wiley   +1 more source

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