Article

Drug Repurposing Screen Identifies an HRI Activating Compound that Promotes Adaptive Mitochondrial Remodeling in MFN2-deficient Cells

2025-06-26

Abstract excerpt

<h4>SUMMARY</h4> Pathogenic variants in the mitochondrial outer membrane GTPase MFN2 cause the peripheral neuropathy Charcot-Marie-Tooth Type 2A (CMT2A). These mutations disrupt MFN2-dependent regulation of diverse aspects of mitochondrial biology including organelle morphology, motility, mitochondrial-endoplasmic reticulum (ER) contacts (MERCs), and respiratory chain activity. However, no therapies currently exi...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: c7628c8a-5b46-50a0-8ecb-66b4bb20d8c6
DOI: 10.1101/2025.06.23.660251

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Drug Repurposing Screen Identifies an HRI Activating Compound that Promotes Adaptive Mitochondrial Remodeling in MFN2-deficient CellsDOI 10.1101/2025.06.23.660251Read

Pharmacologic Activation of Integrated Stress Response Kinases Inhibits Pathologic Mitochondrial FragmentationSemantic proximity Read

Pseudohypoxia-stabilized HIF2⍺ transcriptionally inhibits MNRR1, a druggable target in MELASSemantic proximity Read

CMT2A-linked MFN2 mutation, T206I promotes mitochondrial hyperfusion and predisposes cells towards mitophagy.Semantic proximity Read

Pathogenic roles of the IMNEPD-associated PTRH2 mutants in aggravating mitochondrial dynamics and its rescue.Semantic proximity Read

Mitochondrial Phenotypes in Genetically Diverse Neurodegenerative Diseases and Their Response to Mitofusin Activation.Semantic proximity Read

Characterization of a novel variant in the HR1 domain of MFN2 in a patient with ataxia, optic atrophy and sensorineural hearing lossSemantic proximity Read

Mitochondrial Nuclear Retrograde Regulator 1 (MNRR1) rescues the cellular phenotype of MELAS by inducing homeostatic mechanisms.Semantic proximity Read

A loss-of-function mutation in the GTPase domain of <i>MFN2</i> , perverting mitochondrial dynamics, is associated with dilated cardiomyopathySemantic proximity Read

Targeting the Mitochondrial Phenotype in Cockayne Syndrome Patient Cells: From Bioenergetic Fragility to Pharmacologic RescueSemantic proximity Read

A novel variant in MFN2 linked to a lethal disorder of neonatal onsetSemantic proximity Read

Endoplasmic Reticulum Alterations in Living Cells Expressing INF2 Variants Causing Glomerulosclerosis and Peripheral NeuropathySemantic proximity Read

Disease-causing MFN2 mutants impair mitochondrial fission dynamics by distinct DRP1 dysregulation.Semantic proximity Read