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D Michel - One of the best experts on this subject based on the ideXlab platform.
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paraneoplastic anti cv2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy
Annals of Neurology, 2001Co-Authors: Jeanchristophe Antoine, Jérôme Honnorat, Jean-philippe Camdessanche, Michel Magistris, Lena Absi, Jeanfrancois Mosnier, P Petiot, Nicolas Kopp, D MichelAbstract:Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd Brain Protein belonging to the family of Ulip/CRMP Proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd Protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study.
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paraneoplastic anti cv2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy
Annals of Neurology, 2001Co-Authors: Jeanchristophe Antoine, Jérôme Honnorat, Jean-philippe Camdessanche, Michel Magistris, Lena Absi, Jeanfrancois Mosnier, P Petiot, Nicolas Kopp, D MichelAbstract:Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd Brain Protein belonging to the family of Ulip/CRMP Proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd Protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study. Ann Neurol 2001;49:214–221
Louis Lim - One of the best experts on this subject based on the ideXlab platform.
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breakpoint cluster region gene product related domain of n chimaerin discrimination between rac binding and gtpase activating residues by mutational analysis
Journal of Biological Chemistry, 1994Co-Authors: Sohail Ahmed, Jiyoung Lee, Longping Wen, Zhuoshen Zhao, A Best, R Kozma, Louis LimAbstract:The breakpoint cluster region gene product (Bcr) is a GTPase-activating Protein (GAP) for members of the Rho family, Cdc42Hs, and Rac1, as is the Brain Protein n-chimaerin. At least 15 Proteins have sequence identity to the GAP domain (150 amino acid residues) of Bcr. The widespread occurrence of Proteins that possess sequence identity to the Bcr-related GAP domain makes it especially important to understand its structure/function relationships. Amino acid sequence alignment of these Proteins reveals three blocks of conservation in the GAP domain. Here, we present a mutational analysis of this domain using n-chimaerin sequences. Ten mutations were constructed (at least two in each of the blocks of conservation), expressed as glutathione S-transferase fusion Proteins in Escherichia coli, and purified. Seven of the mutants, including deletions, still possessed GAP activity for Rac1. Three of the mutants had no Rac1-GAP activity but were still able to bind Rac1. IC50 values obtained from competition experiments suggest that n-chimaerin and the mutants with no GAP activity bound Rac1 with similar apparent binding constants. Thus, this mutant analysis allows discrimination between Rac1-binding and Rac1 GTPase- activating residues.
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the human active breakpoint cluster region related gene encodes a Brain Protein with homology to guanine nucleotide exchange Proteins and gtpase activating Proteins
Journal of Biological Chemistry, 1993Co-Authors: Enechoo Tan, T Leung, E Manser, Louis LimAbstract:GTPase-activating Proteins (GAPs) modulate the activity of the ras superfamily of Proteins by converting active GTP-bound to inactive GDP-bound p21s. Employing a novel GAP overlay assay (Manser, E., Leung, T., Monfries, C., Teo, M., Hall, C., and Lim, L. (1992) J. Biol. Chem. 267, 16025-16028), we demonstrated a diversity of Proteins with GAP activities in different tissues. Using a polymerase chain reaction strategy exploiting conserved residues in the GAP domains of n-chimaerin and the product of the breakpoint cluster region gene (BCR), we isolated a human Brain 5.3-kilobase cDNA containing a 486-base pair region with complete identity to a previously reported active BCR-related (ABR) gene sequence on human chromosome 17. The Brain cDNA encoded a 98-kDa Protein (ABR) resembling BCR (68% identity), containing both the oncogene dbl-related domain at the N terminus and the GAP domain at the C terminus; however, it lacks the N-terminal BCR Protein kinase domain. The ABR GAP domain expressed as an Escherichia coli fusion Protein was active against Rac1 and Cdc42 of the rho subfamily. The ABR mRNA is highly enriched in the Brain. ABR probably corresponds to the Brain-enriched 100-kDa GAP for Rac and Cdc42Hs previously detected. The relationship of ABR to Miller-Dieker syndrome, a neurological disorder co-mapping to 17p13.3, is discussed.
Jeanchristophe Antoine - One of the best experts on this subject based on the ideXlab platform.
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paraneoplastic anti cv2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy
Annals of Neurology, 2001Co-Authors: Jeanchristophe Antoine, Jérôme Honnorat, Jean-philippe Camdessanche, Michel Magistris, Lena Absi, Jeanfrancois Mosnier, P Petiot, Nicolas Kopp, D MichelAbstract:Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd Brain Protein belonging to the family of Ulip/CRMP Proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd Protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study.
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paraneoplastic anti cv2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy
Annals of Neurology, 2001Co-Authors: Jeanchristophe Antoine, Jérôme Honnorat, Jean-philippe Camdessanche, Michel Magistris, Lena Absi, Jeanfrancois Mosnier, P Petiot, Nicolas Kopp, D MichelAbstract:Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd Brain Protein belonging to the family of Ulip/CRMP Proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd Protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study. Ann Neurol 2001;49:214–221
Jianwei Jiao - One of the best experts on this subject based on the ideXlab platform.
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disc1 regulates astrogenesis in the embryonic Brain via modulation of ras mek erk signaling through rassf7
Development, 2016Co-Authors: Shukun Wang, Qingli Liang, Huimin Qiao, Tianjin Shen, Jianwei JiaoAbstract:Disrupted in schizophrenia 1 (DISC1) is known as a high susceptibility gene for schizophrenia. Recent studies have indicated that schizophrenia might be caused by glia defects and dysfunction. However, there is no direct evidence of a link between the schizophrenia gene DISC1 and gliogenesis defects. Thus, an investigation into the involvement of DISC1 (a ubiquitously expressed Brain Protein) in astrogenesis during the late stage of mouse embryonic Brain development is warranted. Here, we show that suppression of DISC1 expression represses astrogenesis in vitro and in vivo, and that DISC1 overexpression substantially enhances the process. Furthermore, mouse and human DISC1 overexpression rescued the astrogenesis defects caused by DISC1 knockdown. Mechanistically, DISC1 activates the RAS/MEK/ERK signaling pathway via direct association with RASSF7. Also, the pERK complex undergoes nuclear translocation and influences the expression of genes related to astrogenesis. In summary, our results demonstrate that DISC1 regulates astrogenesis by modulating RAS/MEK/ERK signaling via RASSF7 and provide a framework for understanding how DISC1 dysfunction might lead to neuropsychiatric diseases.
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disc1 regulates astrogenesis in the embryonic Brain via modulation of ras mek erk signaling through rassf7
bioRxiv, 2015Co-Authors: Shukun Wang, Qingli Liang, Huimin Qiao, Tianjin Shen, Jianwei JiaoAbstract:Disrupted in Schizophrenia1 (DISC1) is known as a high susceptibility gene of schizophrenia. More recent studies have connected schizophrenia with glia defects and dysfunction. However, it is unclear whether there is connection between DISC1 and gliogenesis defect. Thus, a precise understanding of DISC1 (a ubiquitously expressed Brain Protein) on astrogenesis in the late stage of embryonic mouse Brain development needs to be deeply investigated. Here, we show that suppression of DISC1 expression represses astrogenesis in vitro and in vivo, whereas DISC1 overexpression substantially enhances the process. Furthermore, mouse and human DISC1 overexpression rescued astrogenesis defects caused by DISC1 knowndown. Mechanistically, DISC1 activates downstream RAS/MEK/ERK signaling pathway via directly associating with the C terminal domain of RASSF7, a RAS association Protein. Also, the pERK complex undergoes nuclear translocation and influences the expression of genes related to astrogenesis. Briefly, our results demonstrate that DISC1 regulates astrogenesis by modulating RAS/MEK/ERK signaling via RASSF7 and provide a framework for understanding how DISC1 dysfunction leads to disease pathology and may contribute to the neuropsychiatric diseases.
Michael G Harrington - One of the best experts on this subject based on the ideXlab platform.
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the 14 3 3 Brain Protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies
The New England Journal of Medicine, 1996Co-Authors: Gary Hsich, Kimbra Kenney, Clarence J Gibbs, Kelvin H Lee, Michael G HarringtonAbstract:Background There is no practical and reliable premortem test for Creutzfeldt–Jakob disease and the related transmissible spongiform encephalopathies. Two Proteins, designated 130 and 131, which have been detected in low concentrations in cerebrospinal fluid from patients with Creutzfeldt–Jakob disease, appear to be sensitive and specific markers for the disease. Attempts to identify these Proteins, however, have been unsuccessful. We hypothesized that they may be present in the normal Brain. Methods We detected Proteins 130 and 131 in normal human Brain, partially sequenced their amino acids, and found that they matched the Brain Protein known as 14-3-3. We then developed a simple, rapid immunoassay for this Protein and tested it in cerebrospinal fluid samples from 71 humans and 30 animals with spongiform encephalopathies and in control samples from 186 humans and 94 animals. Results The immunoassay detected the 14-3-3 Protein in cerebrospinal fluid from 68 of the 71 patients with Creutzfeldt–Jakob diseas...