Progressive mitochondrial and autophagic dysfunction during RGC development driven by a LHON-associated mitochondrial tRNAThr mutation.

doi:10.1016/j.cellsig.2026.112778

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Community

Back to search

Article

Progressive mitochondrial and autophagic dysfunction during RGC development driven by a LHON-associated mitochondrial tRNAThr mutation.

Cellular signalling - 1 Dec 2026

Guan Wei, Li Wenxu, Li Zijun, Peng Lu, Shao Shidan, Zhang Juanjuan

Abstract excerpt

Leber's hereditary optic neuropathy (LHON) is a genetically inherited disease of the eye triggered by mtDNA mutations, leading to degeneration of RGCs. We previously reported that the mitochondrial tRNAThr (MT-TT) 15927G > A homoplasmic mutation disrupted the base pairing (28C-42G) conserved in the anticodon stem of tRNAThr, impairing t6A modification, aminoacylation, and steady-state tRNAThr levels, ultimately...

Read the complete abstract on PubMed

Topics

Optic Atrophy, Hereditary, Leber
Humans
Mitochondria
Autophagy
Retinal Ganglion Cells
Mutation

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Reactive Oxygen Species

Membrane Potential, Mitochondrial

RNA, Transfer, Thr

Induced Pluripotent Stem Cells

Cell Differentiation

Mitophagy

Mitochondrial Dynamics

DNA, Mitochondrial

Source

Open in PubMed

Identifiers

PMID: 42543077
DOI: 10.1016/j.cellsig.2026.112778

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 23 Oct 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Progressive mitochondrial and autophagic dysfunction during RGC development driven by a LHON-associated mitochondrial tRNAThr mutation.PMID 42543077Read ↗

Nuclear modifier YARS2 allele correction restored retinal ganglion cells-specific deficiencies in Leber's hereditary optic neuropathy.Semantic proximity Read ↗

Abnormal morphology and function in retinal ganglion cells derived from patients-specific iPSCs generated from individuals with Leber's hereditary optic neuropathy.Semantic proximity Read ↗

Mitophagy activation repairs Leber’s hereditary optic neuropathy-associated mitochondrial dysfunction and improves cell survivalSemantic proximity Read ↗

Mitochondrial tRNAGlu 14693A > G Mutation, an "Enhancer" to the Phenotypic Expression of Leber's Hereditary Optic Neuropathy.Semantic proximity Read ↗

Leber hereditary optic neuropathy-new insights and old challenges.Semantic proximity Read ↗

Leber's hereditary optic neuropathy-associated ND6 14484T > C mutation caused pleiotropic effects on the complex I, RNA homeostasis, apoptosis and mitophagy.Semantic proximity Read ↗

Assocation Between Leber's Hereditary Optic Neuropathy and MT-ND1 3460G>A Mutation-Induced Alterations in Mitochondrial Function, Apoptosis, and Mitophagy.Semantic proximity Read ↗

Mesenchymal stem cell mitochondrial transfer effectively protects Leber's Hereditary Optic Neuropathy (LHON) mutant cells from mitochondrial damage.Semantic proximity Read ↗

The pathological mechanisms and novel therapeutics for Leber's hereditary optic neuropathy.Semantic proximity Read ↗

Dysregulation of mitochondria, apoptosis and mitophagy in Leber's hereditary optic neuropathy with MT-ND1 3635G>A mutation.Semantic proximity Read ↗

Pathological mitophagy disrupts mitochondrial homeostasis in Leber's hereditary optic neuropathy.Semantic proximity Read ↗

Mitochondrial replacement in an iPSC model of Leber’s hereditary optic neuropathySemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.