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Article

Autophagy induced by Alexander disease-mutant GFAP accumulation is regulated by p38/MAPK and mTOR signaling pathways.

Human molecular genetics - 1 Jun 2008

Tang Guomei, Yue Zhenyu, Talloczy Zsolt, Hagemann Tracy, Cho Woosung, Messing Albee, Sulzer David L, Goldman James E

Abstract excerpt

Glial fibrillary acidic protein (GFAP) is the principle intermediate filament (IF) protein in astrocytes. Mutations in the GFAP gene lead to Alexander disease (AxD), a rare, fatal neurological disorder characterized by the presence of abnormal astrocytes that contain GFAP protein aggregates, term...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 18276609
DOI: 10.1093/hmg/ddn042
PMCID: PMC2902290

Genes

GFAP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-18276609-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

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Autophagy induced by Alexander disease-mutant GFAP accumulation is regulated by p38/MAPK and mTOR signaling pathways.PMID 18276609Read ↗

Synergistic Effects of Glial Fibrillary Acidic Protein Mutation and Overexpression in the Pathogenesis of Alexander Disease.Semantic proximity Read ↗

GFAP and its role in Alexander disease.Semantic proximity Read ↗

Alexander disease: models, mechanisms, and medicine.Semantic proximity Read ↗

Pexidartinib treatment in Alexander disease model mice reduces macrophage numbers and increases glial fibrillary acidic protein levels, yet has minimal impact on other disease phenotypes.Semantic proximity Read ↗

Glial fibrillary acidic protein is pathologically modified in Alexander disease.Semantic proximity Read ↗

Astrocytic TDP-43 Pathology in Alexander DiseaseSemantic proximity Read ↗

Phenotypic conversions of "protoplasmic" to "reactive" astrocytes in Alexander disease.Semantic proximity Read ↗

Aggregation-prone GFAP mutation in Alexander disease validated using a zebrafish model.Semantic proximity Read ↗

Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander disease.Semantic proximity Read ↗

Elevated GFAP isoform expression promotes protein aggregation and compromises astrocyte function.Semantic proximity Read ↗

Alexander Disease: A Literature Review for Clinicians.Semantic proximity Read ↗

Metabolic Enzyme Alterations and Astrocyte Dysfunction in a Murine Model of Alexander Disease With Severe Reactive Gliosis.Semantic proximity Read ↗

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