GFAP in health and disease

J Middeldorp, EM Hol - Progress in neurobiology, 2011 - Elsevier
Glial fibrillary acidic protein (GFAP) is the main intermediate filament protein in mature
astrocytes, but also an important component of the cytoskeleton in astrocytes during …

Intermediate filaments: from cell architecture to nanomechanics

H Herrmann, H Bär, L Kreplak, SV Strelkov… - … reviews Molecular cell …, 2007 - nature.com
Intermediate filaments (IFs) constitute a major structural element of animal cells. They build
two distinct systems, one in the nucleus and one in the cytoplasm. In both cases, their major …

Crystallins in the eye: function and pathology

UP Andley - Progress in retinal and eye research, 2007 - Elsevier
Crystallins are the predominant structural proteins in the lens that are evolutionarily related
to stress proteins. They were first discovered outside the vertebrate eye lens by Bhat and …

Neurological diseases as primary gliopathies: a reassessment of neurocentrism

A Verkhratsky, MV Sofroniew, A Messing… - ASN …, 2012 - journals.sagepub.com
Diseases of the human brain are almost universally attributed to malfunction or loss of nerve
cells. However, a considerable amount of work has, during the last decade, expanded our …

GFAP and its role in Alexander disease

RA Quinlan, M Brenner, JE Goldman… - Experimental cell research, 2007 - Elsevier
Here we review how GFAP mutations cause Alexander disease. The current data suggest
that a combination of events cause the disease. These include:(i) the accumulation of GFAP …

[PDF][PDF] Autophagy activation by rapamycin eliminates mouse Mallory‐Denk bodies and blocks their proteasome inhibitor‐mediated formation

M Harada, S Hanada, DM Toivola, N Ghori… - …, 2008 - Wiley Online Library
The proteasomal and lysosomal/autophagy pathways in the liver and other tissues are
involved in several biological processes including the degradation of misfolded proteins …

[HTML][HTML] Alexander disease GFAP R239C mutant shows increased susceptibility to lipoxidation and elicits mitochondrial dysfunction and oxidative stress

Á Viedma-Poyatos, P González-Jiménez, MA Pajares… - Redox Biology, 2022 - Elsevier
Alexander disease is a fatal neurological disorder caused by mutations in the intermediate
filament protein Glial Fibrillary Acidic Protein (GFAP), which is key for astrocyte homeostasis …

Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander disease severity

RA Battaglia, AS Beltran, S Delic, R Dumitru… - Elife, 2019 - elifesciences.org
Alexander disease (AxD) is a fatal neurodegenerative disorder caused by mutations in glial
fibrillary acidic protein (GFAP), which supports the structural integrity of astrocytes. Over 70 …

Cortical expression of glial fibrillary acidic protein and glutamine synthetase is decreased in schizophrenia

AE Steffek, RE McCullumsmith, V Haroutunian… - Schizophrenia …, 2008 - Elsevier
Altered expression of structural and functional molecules expressed by astrocytes may play
a role in the pathophysiology of schizophrenia. We investigated the hypothesis that the …

Disruption of neurofilament network with aggregation of light neurofilament protein: a common pathway leading to motor neuron degeneration due to Charcot–Marie …

J Zhai, H Lin, JP Julien… - Human molecular …, 2007 - academic.oup.com
Mutations in neurofilament light (NFL) subunit and small heat-shock protein B1 (HSPB1)
cause autosomal-dominant axonal Charcot–Marie–Tooth disease type 2E (CMT2E) and …