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Article

Activation of the Unfolded Protein Response in Sporadic Inclusion-Body Myositis but Not in Hereditary GNE Inclusion-Body Myopathy.

Journal of neuropathology and experimental neurology - 1 Jun 2015

Nogalska Anna, D'Agostino Carla, Engel W King, Cacciottolo Mafalda, Asada Shinichi, Mori Kazutoshi, Askanas Valerie

Abstract excerpt

Muscle fibers in patients with sporadic inclusion-body myositis (s-IBM),the most common age-associated myopathy, are characterized by autophagic vacuoles and accumulation of ubiquitinated and congophilic multiprotein aggregates that contain amyloid-β and phosphorylated tau. Muscle fibers of autosomal-recessive hereditary inclusion-body myopathy caused by the GNE mutation (GNE-h-IBM) display similar pathologic...

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Source

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Identifiers

PMID: 25978849
DOI: 10.1097/NEN.0000000000000196
PMCID: PMC4506930

Genes

GNE
ATF6
ATF4
EBP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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