Autoimmune glial fibrillary acidic protein astrocytopathy resembling isolated central nervous system lymphomatoid granulomatosis

2021 ◽  
pp. 577748
Author(s):  
Akio Kimura ◽  
Shinei Kato ◽  
Akira Takekoshi ◽  
Nobuaki Yoshikura ◽  
Narufumi Yanagida ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Acidic Protein ◽  
Lymphomatoid Granulomatosis

scholarly journals Refining the concept of GFAP toxicity in Alexander disease

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Albee Messing
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Intermediate Filament ◽  
Alexander Disease ◽  
Acidic Protein ◽  
Sequence Variants ◽  
Main Body ◽  
Gain Of Function ◽  
The Central Nervous System

Abstract Background Alexander disease is caused by dominantly acting mutations in glial fibrillary acidic protein (GFAP), the major intermediate filament of astrocytes in the central nervous system. Main body In addition to the sequence variants that represent the origin of disease, GFAP accumulation also takes place, together leading to a gain-of-function that has sometimes been referred to as “GFAP toxicity.” Whether the nature of GFAP toxicity in patients, who have mixtures of both mutant and normal protein, is the same as that produced by simple GFAP excess, is not yet clear. Conclusion The implications of these questions for the design of effective treatments are discussed.

scholarly journals Abnormal Reaction to Central Nervous System Injury in Mice Lacking Glial Fibrillary Acidic Protein and Vimentin

1999 ◽  
Vol 145 (3) ◽  
pp. 503-514 ◽  
Author(s):  
Milos Pekny ◽  
Clas B. Johansson ◽  
Camilla Eliasson ◽  
Josefina Stakeberg ◽  
Åsa Wallén ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Intermediate Filament ◽  
Glial Scar ◽  
Scar Formation ◽  
Brain Lesions ◽  
Acidic Protein ◽  
The Central Nervous System

In response to injury of the central nervous system, astrocytes become reactive and express high levels of the intermediate filament (IF) proteins glial fibrillary acidic protein (GFAP), vimentin, and nestin. We have shown that astrocytes in mice deficient for both GFAP and vimentin (GFAP−/−vim−/−) cannot form IFs even when nestin is expressed and are thus devoid of IFs in their reactive state. Here, we have studied the reaction to injury in the central nervous system in GFAP−/−, vimentin−/−, or GFAP−/−vim−/− mice. Glial scar formation appeared normal after spinal cord or brain lesions in GFAP−/− or vimentin−/− mice, but was impaired in GFAP−/−vim−/− mice that developed less dense scars frequently accompanied by bleeding. These results show that GFAP and vimentin are required for proper glial scar formation in the injured central nervous system and that some degree of functional overlap exists between these IF proteins.

scholarly journals Immunohistochemical expression of glial fibrillary acidic protein and CAM5.2 in glial tumors and their role in differentiating glial tumors from metastatic tumors of central nervous system

2015 ◽  
Vol 06 (04) ◽  
pp. 499-503 ◽  
Author(s):  
Rama Goyal ◽  
Satyavir Kumar Mathur ◽  
Sumiti Gupta ◽  
Rahul Goyal ◽  
Sanjay Kumar ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glial Fibrillary Acidic Protein ◽  
Metastatic Carcinoma ◽  
Acidic Protein ◽  
Histopathological Diagnosis ◽  
Immune Markers ◽  
Metastatic Tumors ◽  
Glial Tumors ◽  
Hematoxylin And Eosin

ABSTRACT Background and Objectives: Immunohistochemistry (IHC) has become an important tool in the diagnosis of metastatic brain tumors. The judicious use of a panel of selected immunostains is unquestionably helpful in diagnostically challenging cases. In our study, the best combination of immune markers useful in differentiating metastatic carcinoma from high-grade gliomas in central nervous system (CNS) are glial fibrillary acidic protein (GFAP) and cytokeratin (CK) (CAM5.2). Materials and Methods: The study was conducted on 80 cases of glial tumors including metastatic tumors to the CNS. Histopathological diagnosis was established on routine hematoxylin and eosin staining of the sections. Special IHC markers, GFAP, and CAM5.2 were used to differentiate glial from metastatic tumors. Result: Of total 80 cases, 40 cases of astrocytic tumors, 2 cases of ependymoma, 2 cases of mixed glial tumors, and 16 cases of glioblastoma multiforme were positive for GFAP. Twelve cases of oligodendroglioma were negative for GFAP. The sensitivity of GFAP in glial tumors was statistically significant as 81.1% and specificity 100%, whereas sensitivity and specificity of CAM5.2 in metastatic tumors were 100%. Conclusion: IHC plays an important role in diagnosing tumors of CNS and markers such as GFAP and CK (CAM5.2) are quite effective in differentiating glial tumors from metastatic tumors of CNS.

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