Alexander Disease Mutations Produce Cells with Coexpression of Glial Fibrillary Acidic Protein and NG2 in Neurosphere Cultures and Inhibit Differentiation into Mature Oligodendrocytes

La alteración de la mielina en la enfermedad de Alexander

(2017) U. Gómez-Pinedo et al.   NEUROLOGIA

Erratum to: Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes

(2016) Takayuki Kondo et al.   Acta Neuropathologica Communications

Astrocyte pathology in Alexander disease causes a marked inflammatory environment

(2015) Markel Olabarria et al.   ACTA NEUROPATHOLOGICA

Glial fibrillary acidic protein: from intermediate filament assembly and gliosis to neurobiomarker

(2015) Zhihui Yang et al.   TRENDS IN NEUROSCIENCES

Adaptive autophagy in Alexander disease-affected astrocytes

(2014) Guomei Tang et al.   Autophagy

Effects of traumatic brain injury on reactive astrogliosis and seizures in mouse models of Alexander disease

(2014) Maria Luisa Cotrina et al.   BRAIN RESEARCH

Histone acetylation in astrocytes suppresses GFAP and stimulates a reorganization of the intermediate filament network

(2014) Regina Kanski et al.   JOURNAL OF CELL SCIENCE

Caspase Cleavage of GFAP Produces an Assembly-Compromised Proteolytic Fragment that Promotes Filament Aggregation

(2013) Mei-Hsuan Chen et al.   ASN Neuro

Adult-onset Alexander disease, associated with a mutation in an alternative GFAP transcript, may be phenotypically modulated by a non-neutral HDAC6 variant

(2013) Laura Melchionda et al.   Orphanet Journal of Rare Diseases

Splice site, frameshift, and chimericGFAPmutations in Alexander disease

(2012) Daniel Flint et al.   HUMAN MUTATION

Beneficial Effects of Nrf2 Overexpression in a Mouse Model of Alexander Disease

(2012) C. M. LaPash Daniels et al.   JOURNAL OF NEUROSCIENCE

Genetic Ablation of Nrf2/Antioxidant Response Pathway in Alexander Disease Mice Reduces Hippocampal Gliosis but Does Not Impact Survival

(2012) Tracy L. Hagemann et al.   PLoS One

Developmental Splicing Deregulation in Leukodystrophies Related to EIF2B Mutations

(2012) Aurélia Huyghe et al.   PLoS One

Serial MRI changes in a patient with infantile Alexander disease and prolonged survival

(2010) Takashi Shiihara et al.   BRAIN & DEVELOPMENT

Glioma Cell Populations Grouped by Different Cell Type Markers Drive Brain Tumor Growth

(2010) Lars Prestegarden et al.   CANCER RESEARCH

In vitro treatments with ceftriaxone promote elimination of mutant glial fibrillary acidic protein and transcription down-regulation

(2010) Tiziana Bachetti et al.   EXPERIMENTAL CELL RESEARCH

Alteration of glial-neuronal metabolic interactions in a mouse model of Alexander disease

(2010) Tore Wergeland Meisingset et al.   GLIA

Drug screening to identify suppressors of GFAP expression

(2010) Woosung Cho et al.   HUMAN MOLECULAR GENETICS

Oligomers of Mutant Glial Fibrillary Acidic Protein (GFAP) Inhibit the Proteasome System in Alexander Disease Astrocytes, and the Small Heat Shock Protein αB-Crystallin Reverses the Inhibition

(2010) Guomei Tang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Defective Glial Maturation in Vanishing White Matter Disease

(2010) Marianna Bugiani et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Suppression of GFAP toxicity by αB-crystallin in mouse models of Alexander disease

(2009) Tracy L. Hagemann et al.   HUMAN MOLECULAR GENETICS

Dysfunctions of neuronal and glial intermediate filaments in disease

(2009) Ronald K.H. Liem et al.   JOURNAL OF CLINICAL INVESTIGATION

The process of inducing GFAP aggregates in astrocytoma-derived cells is different between R239C and R416W mutant GFAP. A time-lapse recording study

(2009) Tomokatsu Yoshida et al.   NEUROSCIENCE LETTERS

Properties of astrocytes cultured from GFAP over-expressing and GFAP mutant mice

(2008) Woosung Cho et al.   EXPERIMENTAL CELL RESEARCH

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

(2008) G. Tang et al.   HUMAN MOLECULAR GENETICS

Glial Fibrillary Acidic Protein Filaments Can Tolerate the Incorporation of Assembly-compromised GFAP-δ, but with Consequences for Filament Organization and αB-Crystallin Association

(2008) Ming-Der Perng et al.   MOLECULAR BIOLOGY OF THE CELL