Article

Microglia from Friedreich Ataxia patients are intrinsically primed for neuroinflammation

2026-07-21

Abstract excerpt

Friedreich Ataxia (FRDA) is an autosomal recessive neurodegenerative disorder characterized by progressive loss of cerebellar and proprioceptive neurons that control movement and coordination. In most patients, FRDA is caused by homozygous GAA trinucleotide repeat expansions in the first intron of the frataxin ( FXN ) gene, resulting in reduced expression of frataxin, a mitochondrial protein essential for biogen...

Topics

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

Identifiers and source

Literature Corpus work: 27031acd-579c-5f7f-8b4e-11e443610b1e
DOI: 10.64898/2026.07.16.738960

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Microglia from Friedreich Ataxia patients are intrinsically primed for neuroinflammationDOI 10.64898/2026.07.16.738960Read

Neuroinflammation in Friedreich’s AtaxiaSemantic proximity Read

Mutant ATXN1 impacts human and mouse microglia and contributes to cognitive, mood, and motor deficits in SCA1 miceSemantic proximity Read

Cell-autonomous immune dysfunction driven by disrupted autophagy in C9orf72 -ALS iPSC-derived microglia contributes to neurodegenerationSemantic proximity Read

Cell-autonomous immune dysfunction driven by disrupted autophagy in <i>C9orf72</i> -ALS iPSC-derived microglia contributes to neurodegenerationSemantic proximity Read

ATM-deficiency induced microglial activation promotes neurodegeneration in Ataxia-TelangiectasiaSemantic proximity Read

Moderate intrinsic phenotypic alterations in C9orf72 ALS/FTD iPSC-microglia despite the presence of C9orf72 pathological featuresSemantic proximity Read

Frataxin deficiency in proprioceptive neurons is causal to inflammatory and glial responses in dorsal root gangliaSemantic proximity Read

Aberrant immunomodulatory signature in β-propeller protein-associated neurodegeneration patient iPSC-derived microglia.Semantic proximity Read

Multiomic profiling reveals aberrant immunomodulatory signature in β-propeller protein-associated neurodegeneration patient iPSC-derived microgliaSemantic proximity Read

Cellular and molecular phenotypes of <i>C9orf72</i> ALS/FTD patient derived iPSC-microglia mono-culturesSemantic proximity Read

C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9Semantic proximity Read

Glial cell activation precedes neurodegeneration in the cerebellar cortex of the YG8-800 murine model of Friedreich’s ataxiaSemantic proximity Read