Discover Community Create You

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

Article

CHCHD10P80L knock-in zebrafish display a mild ALS-like phenotype.

Experimental neurology - 1 Dec 2024

Petel Légaré Virginie, Harji Ziyaan A, Rampal Christian J, Antonicka Hana, Gurberg Tyler J N, Persia Olivia, Rodríguez Esteban C, Shoubridge E A, Armstrong Gary A B

Abstract excerpt

Mutations in the nuclear-encoded mitochondrial gene CHCHD10 have been observed in patients with a spectrum of diseases that include amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). To investigate the pathogenic nature of disease-associated variants of CHCHD10 we generated a zebrafish knock-in (KI) model expressing the orthologous ALS-associated CHCHD10P80L variant (zebrafish: Chchd10P83L)....

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Amyotrophic Lateral Sclerosis
Share to Animals, Genetically Modified

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gene Knock-In Techniques

Share to Mitochondrial Proteins

Share to Motor Neurons

Share to Neuromuscular Junction

Share to Zebrafish

Share to Zebrafish Proteins

Source

Identifiers

PMID: 39260590
DOI: 10.1016/j.expneurol.2024.114945

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

CHCHD10P80L knock-in zebrafish display a mild ALS-like phenotype.PMID 39260590Read ↗

Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mt-ISRSemantic proximity Read ↗

Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response.Semantic proximity Read ↗

Genetic and immunopathological analysis of CHCHD10 in Australian amyotrophic lateral sclerosis and frontotemporal dementia and transgenic TDP-43 mice.Semantic proximity Read ↗

CHCHD10 mutations induce tissue-specific mitochondrial DNA deletions with a distinct signature.Semantic proximity Read ↗

CHCHD2 and CHCHD10-related neurodegeneration: molecular pathogenesis and the path to precision therapy.Semantic proximity Read ↗

Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrity.Semantic proximity Read ↗

Early death of ALS-linked CHCHD10-R15L transgenic mice with central nervous system, skeletal muscle, and cardiac pathologySemantic proximity Read ↗

TARDBP (TDP-43) Knock-in Zebrafish Display a Late-Onset Motor Phenotype and Loss of Large Spinal Cord Motor Neurons.Semantic proximity Read ↗

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouseSemantic proximity Read ↗

Mutation analysis of<i>CHCHD10</i>in different neurodegenerative diseasesSemantic proximity Read ↗

Modulation of synaptic plasticity, motor unit physiology, and TDP-43 pathology by CHCHD10Semantic proximity Read ↗

OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy.Semantic proximity Read ↗

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