The Experts below are selected from a list of 72 Experts worldwide ranked by ideXlab platform
James E Goldman - One of the best experts on this subject based on the ideXlab platform.
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overexpression and abnormal modification of the stress proteins alpha b crystallin and hsp27 in alexander disease
American Journal of Pathology, 1993Co-Authors: Mark Head, E Corbin, James E GoldmanAbstract:Abstract Alexander disease is a leukodystrophy characterized by the presence of numerous Rosenthal Fibers, inclusion bodies in astrocytes. A major component of Rosenthal Fibers is alpha B-crystallin, some of which is ubiquitinated. In this report, we show that Alexander central nervous system (CNS) tissues contain elevated messenger RNA and protein levels of both alpha B-crystallin and the related small heat shock protein, hsp27, and that Rosenthal Fibers contain hsp27. The alpha B-crystallin and hsp27 polypeptide isoform patterns of Alexander disease CNS are also distinct from those of control samples, suggesting that postranslational modifications may be involved in Rosenthal Fiber formation. We advance the hypothesis that Rosenthal Fibers may be regarded as stress protein inclusions formed in astrocytes as part of a chronic stress response to an as yet unknown stimulus in the CNS of Alexander patients.
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αB-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal Fibers
The American journal of pathology, 1993Co-Authors: Toru Iwaki, Akiko Iwaki, Jun Tateishi, Yoshiyuki Sakaki, James E GoldmanAbstract:To understand the significance of the accumulation of alpha B-crystallin in Rosenthal Fibers within astrocytes, the expression and metabolism of alpha B-crystallin in glioma cell lines were examined under the conditions of heat and oxidative stress. alpha B-crystallin mRNA was increased after both stresses, and alpha B-crystallin protein moved from a detergent-soluble to a detergent-insoluble form. In addition, Western blotting of Alexander's disease brain homogenates revealed that the 27-kd heat shock protein (HSP27), which is related to alpha B-crystallin, accumulates along with alpha B-crystallin. The presence of HSP27 in Rosenthal Fibers was directly demonstrated by immunohistochemistry. Our results suggest that astrocytes in Alexander's disease may be involved in an as yet unknown kind of stress reaction that causes the accumulation of alpha B-crystallin and HSP27 and results in Rosenthal Fiber formation.
Harry V. Vinters - One of the best experts on this subject based on the ideXlab platform.
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Cortical dysplasia with prominent Rosenthal Fiber formation in a case of intractable pediatric epilepsy.
Human pathology, 2009Co-Authors: Negar Khanlou, Gary W. Mathern, Wendy G. Mitchell, Noriko Salamon, Whitney B. Pope, William H. Yong, Harry V. VintersAbstract:We report a case of a 5-year-old boy with intractable epilepsy who underwent therapeutic corticectomy. Histopathologic findings within the resection specimen included severe cortical dysplasia associated with abundant subpial and intraparenchymal Rosenthal Fibers in a large right frontal lesion that merged into the basal ganglia. Rosenthal Fiber proliferation may represent a reactive process, are frequent in pilocytic astrocytomas, and are a defining feature of Alexander disease. There was no evidence of neoplasm or leukodystrophy in this case. Genetic analysis of the specimen showed a few previously reported polymorphisms but no mutation in the GFAP gene. This case is unique among several hundred cortical resection specimens that we have studied, including numerous cases of severe cortical dysplasia.
Toru Iwaki - One of the best experts on this subject based on the ideXlab platform.
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αB-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal Fibers
The American journal of pathology, 1993Co-Authors: Toru Iwaki, Akiko Iwaki, Jun Tateishi, Yoshiyuki Sakaki, James E GoldmanAbstract:To understand the significance of the accumulation of alpha B-crystallin in Rosenthal Fibers within astrocytes, the expression and metabolism of alpha B-crystallin in glioma cell lines were examined under the conditions of heat and oxidative stress. alpha B-crystallin mRNA was increased after both stresses, and alpha B-crystallin protein moved from a detergent-soluble to a detergent-insoluble form. In addition, Western blotting of Alexander's disease brain homogenates revealed that the 27-kd heat shock protein (HSP27), which is related to alpha B-crystallin, accumulates along with alpha B-crystallin. The presence of HSP27 in Rosenthal Fibers was directly demonstrated by immunohistochemistry. Our results suggest that astrocytes in Alexander's disease may be involved in an as yet unknown kind of stress reaction that causes the accumulation of alpha B-crystallin and HSP27 and results in Rosenthal Fiber formation.
Negar Khanlou - One of the best experts on this subject based on the ideXlab platform.
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Cortical dysplasia with prominent Rosenthal Fiber formation in a case of intractable pediatric epilepsy.
Human pathology, 2009Co-Authors: Negar Khanlou, Gary W. Mathern, Wendy G. Mitchell, Noriko Salamon, Whitney B. Pope, William H. Yong, Harry V. VintersAbstract:We report a case of a 5-year-old boy with intractable epilepsy who underwent therapeutic corticectomy. Histopathologic findings within the resection specimen included severe cortical dysplasia associated with abundant subpial and intraparenchymal Rosenthal Fibers in a large right frontal lesion that merged into the basal ganglia. Rosenthal Fiber proliferation may represent a reactive process, are frequent in pilocytic astrocytomas, and are a defining feature of Alexander disease. There was no evidence of neoplasm or leukodystrophy in this case. Genetic analysis of the specimen showed a few previously reported polymorphisms but no mutation in the GFAP gene. This case is unique among several hundred cortical resection specimens that we have studied, including numerous cases of severe cortical dysplasia.
Akiko Iwaki - One of the best experts on this subject based on the ideXlab platform.
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αB-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal Fibers
The American journal of pathology, 1993Co-Authors: Toru Iwaki, Akiko Iwaki, Jun Tateishi, Yoshiyuki Sakaki, James E GoldmanAbstract:To understand the significance of the accumulation of alpha B-crystallin in Rosenthal Fibers within astrocytes, the expression and metabolism of alpha B-crystallin in glioma cell lines were examined under the conditions of heat and oxidative stress. alpha B-crystallin mRNA was increased after both stresses, and alpha B-crystallin protein moved from a detergent-soluble to a detergent-insoluble form. In addition, Western blotting of Alexander's disease brain homogenates revealed that the 27-kd heat shock protein (HSP27), which is related to alpha B-crystallin, accumulates along with alpha B-crystallin. The presence of HSP27 in Rosenthal Fibers was directly demonstrated by immunohistochemistry. Our results suggest that astrocytes in Alexander's disease may be involved in an as yet unknown kind of stress reaction that causes the accumulation of alpha B-crystallin and HSP27 and results in Rosenthal Fiber formation.
