Article

Fructose-2,6-bisphosphate restores DNA repair activity of PNKP and ameliorates neurodegenerative symptoms in Huntington’s disease

2023-10-26

Abstract excerpt

Huntington’s disease (HD) and spinocerebellar ataxia type 3 (SCA3) are the two most prevalent polyglutamine (polyQ) neurodegenerative diseases, caused by CAG (encoding glutamine) repeat expansion in the coding region of the huntingtin (HTT) and ataxin-3 (ATXN3) proteins, respectively. We have earlier reported that the activity, but not the protein level, of an essential DNA repair enzyme, polynucleotide kinase 3’-...

Topics

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

Identifiers and source

Literature Corpus work: bf75edd4-e98d-59b6-983c-76b2a50bef34
DOI: 10.1101/2023.10.26.564220

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Fructose-2,6-bisphosphate restores DNA repair activity of PNKP and ameliorates neurodegenerative symptoms in Huntington’s diseaseDOI 10.1101/2023.10.26.564220Read

F2,6BP restores mitochondrial genome integrity in Huntington’s DiseaseSemantic proximity Read

Fructose-2,6-bisphosphate restores TDP-43 pathology-driven genome repair deficiency in motor neuron diseasesSemantic proximity Read

A Toxic Tau-PFKFB3 Circuit Reduces F2,6BP Levels and Drives NeurodegenerationSemantic proximity Read

The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3'-phosphatase in spinocerebellar ataxia type 3 pathogenesis.Semantic proximity Read

Longevity-Associated Variant of BPIFB4 Confers Neuroprotection in the STHdh Cell Model of Huntington Disease.Semantic proximity Read

Deficiency in classical nonhomologous end-joining-mediated repair of transcribed genes is linked to SCA3 pathogenesis.Semantic proximity Read

Direct Measure of DNA Damage in Huntington Disease Reveals Elevated Oxidative Genotoxic Stress and Dysfunctional DNA RepairSemantic proximity Read

Targeting PFKFB3 alleviates cerebral ischemia-reperfusion injury in miceSemantic proximity Read

Huntingtin interactome reveals huntingtin role in regulation of double strand break DNA damage response (DSB/DDR), chromatin remodeling and RNA processing pathwaysSemantic proximity Read

Pathological mutations in PNKP trigger defects in DNA single-strand break repair but not DNA double-strand break repair.Semantic proximity Read

Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice.Semantic proximity Read

Mutations of the Huntington's disease protein impact on the ATM-dependent signaling and repair pathways of the radiation-induced DNA double-strand breaks: corrective effect of statins and bisphosphonates.Semantic proximity Read