All forms of Alexander disease are characterized by the presence of Rosenthal

bodies, Rosenthal fibers, hemosiderin, and areas of calcification are often noted.

Oct 23, 2006 . Fluoro Jade co-labeled tissue samples stained with GFAP immunofluorescence.

Rosenthal fibers are densely compacted masses of glial intermediate filaments

Owing to the discovery of inclusion bodies indistinguishable from Rosenthal

Rosenthal fibers were easily recognized throughout the tumor including the

the gene that makes glial fibrillary acidic protein (GFAP). GFAP is found in

The researchers found that if they prevented vimentin from forming, even normal

The particular GFAP mutation present does not appear to have any effect on the

Correspondence to Nilika Shah Singhal: singhaln@neuropeds.ucsf.edu.

formation of astrocytic cytoplasmic inclusions called. Rosenthal fibers, which

The Alexander disease-causing glial fibrillary acidic protein mutant, R416W,

Oct 25, 2006 . Alexander Disease-Associated Glial Fibrillary Acidic Protein Mutations in Mice

The cellular mechanism of the disease is the presence of cytoplasmic

Showing a heterozygous mutation in the glial fibrillary acidic protein (GFAP) gene

[4] All forms of Alexander's disease are characterized by the presence of

Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with .

Jul 13, 2005 . To study further the effects of elevated GFAP and Rosenthal fibers per se,

nated proteins are termed Rosenthal fibers and characterize. Alexander disease,

. white matter degeneration and cytoplasmic inclusions in astrocytes called

All forms of Alexander disease are characterized by the presence of Rosenthal

[Immunohistochemical study on Rosenthal fibers in gliomas using anti-GFAP and

Aug 1, 2006 . The Alexander disease-causing Glial Fibrillary Acidic Protein mutant, R416W,

Keywords. Alexander's disease, astrocyte, Rosenthal fibers, glial fibrillary acidic

Mar 17, 2008 . inclusions, called Rosenthal fibers, which contain GFAP and small heat shock

Rosenthal fibre immunoelectron microscopy. 457. PBS (0.05 M pH 7.4-7.6) at

Jun 23, 2008 . The Alexander disease-causing Glial Fibrillary Acidic Protein mutant, R416W,

Jun 12, 2006 . These aggregates, termed “Rosenthal fibers,” contain the protein chaperones αB-

to Rosenthal fibers, the hallmark feature of Alexander disease (Messing et al.,

Oct 12, 2011 . GFAP is a filamentous protein of astrocytes and also accumulates as part of the

Alexander's disease, a leukodystrophy characterized by Rosenthal fibers (RFs) in

Hagemann TL, Connor JX, & Messing A. Alexander disease-associated GFAP

View Gfap allele: origin, molecular description, curated phenotype details and .

The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with

The chance discovery that mice expressing a human GFAP transgene formed

Ultrastructural immunocytochemistry revealed that the Rosenthal fibers contained

All forms of Alexander disease are characterized by the presence of Rosenthal

Most cases have been shown to be due to mutations in the gene encoding glial

Jan 13, 2011 . Fig. 4. Cerebellum, sheep no. 2. Rosenthal fibers have peripheral GFAP

Rosenthal fibers consist of two components: bundles of intermediate filaments,

Astrocytes cultured from transgenic mice carrying the added human glial fibrillary

Alexander Disease-Associated Glial Fibrillary Acidic Protein. Mutations in Mice

Aug 1, 2006 . The Alexander Disease–Causing Glial Fibrillary Acidic Protein Mutant, R416W,

Jan 7, 2009 . These mutations lead to protein aggregation and formation of Rosenthal fibers,

Paper-894431. When mice with Rosenthal fibers induced by over-expression of

Intermediate Filaments and GFAP. A deeper understanding of Rosenthal fibers is

Jan 28, 2010 . No Rosenthal fibers are present in control white matter. GFAP immunostaining of

Ultrastructural immunoreactivities of alpha B-crystallin, glial fibrillary acidic

May 1, 2005. causes filament disorganization and decreased solubility of GFAP . called

Rosenthal fibers share epitopes with alpha B-crystallin, glial fibrillary acidic

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