Literature DB >> 1407707

Accumulation of alpha B-crystallin in brains of patients with Alexander's disease is not due to an abnormality of the 5'-flanking and coding sequence of the genomic DNA.

A Iwaki1, T Iwaki, J E Goldman, K Ogomori, J Tateishi, Y Sakaki.   

Abstract

alpha B-Crystallin is a major protein component of Rosenthal fibers, which massively accumulate in the brains of patients suffering from Alexander's disease. To examine whether or not accumulation of alpha B-crystallin is due to any abnormality of the gene structures, we determined the sequence of the alpha B-crystallin gene in two cases of pathologically confirmed Alexander's disease. Direct sequencing of the promoter and coding regions of the alpha B-crystallin gene in patients revealed them to have a normal sequence. Northern blotting showed a single alpha B-crystallin mRNA species expressed in the Alexander's disease brain.

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Year:  1992        PMID： 1407707     DOI： 10.1016/0304-3940(92)90689-5

Source DB:  PubMed          Journal:  Neurosci Lett        ISSN： 0304-3940            Impact factor:   3.046


  9 in total

1.  Juvenile alexander disease: a case report.

Authors:  Halit Ozkaya; Abdullah Baris Akcan; Gokhan Aydemir; Mustafa Kul; Secil Aydinoz; Ferhan Karademir; Selami Suleymanoglu
Journal:  Eurasian J Med       Date:  2012-04

2.  The Alexander disease-causing glial fibrillary acidic protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alpha B-crystallin and HSP27.

Authors:  Ming Der Perng; Mu Su; Shu Fang Wen; Rong Li; Terry Gibbon; Alan R Prescott; Michael Brenner; Roy A Quinlan
Journal:  Am J Hum Genet       Date:  2006-06-12       Impact factor: 11.025

3.  Alpha B-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal fibers.

Authors:  T Iwaki; A Iwaki; J Tateishi; Y Sakaki; J E Goldman
Journal:  Am J Pathol       Date:  1993-08       Impact factor: 4.307

4.  Alexander's disease in a Bernese mountain dog.

Authors:  H Weissenböck; G Obermaier; E Dahme
Journal:  Acta Neuropathol       Date:  1996       Impact factor: 17.088

5.  Global expression profiling identifies a novel biosignature for protein aggregation R120GCryAB cardiomyopathy in mice.

Authors:  Namakkal S Rajasekaran; Matthew A Firpo; Brett A Milash; Robert B Weiss; Ivor J Benjamin
Journal:  Physiol Genomics       Date:  2008-07-15       Impact factor: 3.107

6.  Alexander disease: a leukodystrophy caused by a mutation in GFAP.

Authors:  Anne B Johnson
Journal:  Neurochem Res       Date:  2004-05       Impact factor: 3.996

7.  Overexpression and abnormal modification of the stress proteins alpha B-crystallin and HSP27 in Alexander disease.

Authors:  M W Head; E Corbin; J E Goldman
Journal:  Am J Pathol       Date:  1993-12       Impact factor: 4.307

8.  Differences in properties between human alphaA- and alphaB-crystallin proteins expressed in Escherichia coli cells in response to cold and extreme pH.

Authors:  Satoru Takeuchi; Yumi Mandai; Akiko Otsu; Taro Shirakawa; Katsuyoshi Masuda; Masanobu Chinami
Journal:  Biochem J       Date:  2003-10-15       Impact factor: 3.857

9.  Atypical MRI features in familial adult onset Alexander disease: case report.

Authors:  Yonghong Liu; Heng Zhou; Huabing Wang; Xiaoqing Gong; Anna Zhou; Lin Zhao; Xindi Li; Xinghu Zhang
Journal:  BMC Neurol       Date:  2016-11-04       Impact factor: 2.474
  9 in total

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