Expression of collagen XVII and ubiquitin-binding protein p62 in motor neuron disease
Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related chang...
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Published in | Brain research Vol. 1247; pp. 171 - 177 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
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Elsevier B.V
09.01.2009
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Abstract | Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease. |
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AbstractList | Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease.Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease. Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease. Abstract Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease. Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in the human brain that include those damaged in motor neuron disease. In this study, we examined the extent of motor neuron disease-related changes in the brain of 9 subjects using ubiquitin-binding protein p62/sequestosome 1 (p62) immunohistochemistry. We then assessed whether or not the expression of collagen XVII was altered in relation to the p62 immunoreactive lesions. We found that neuronal collagen XVII expression in motor neuron disease remains similar to that seen in the normal human brain and thus a change in collagen XVII expression is not an immunohistochemically detectable feature of motor neuron disease. We also found that the regional distribution of p62 varied according to clinical presentation: p62 immunoreactive inclusions were found in the frontal cortex, hippocampus and cerebellum only in subjects with a history of psychiatric morbidity. Our study supports the re-definition of motor neuron disease as a multisystem disorder with a wide clinicopathological spectrum, and we advocate addressing psychiatric symptomology in future studies of motor neuron disease. degeneration with ubiquitinated inclusions and amyotrophic lateral sclerosis, IHC, immunohistochemistry 43 |
Author | Hofmann, Silke C. Majamaa, Kari Hartikainen, Päivi Pikkarainen, Maria Alafuzoff, Irina Seppänen, Allan |
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Keywords | coll XVII MND NCI PMA Collagen XVII NT TDP-43 Motor neuron disease ALS CNS IR p62 motor neuron disease neuronal cytoplasmic inclusions progressive muscular atrophy amyotrophic lateral sclerosis central nervous system immunoreactive trans-activating responsive sequence DNA-binding protein 43 neurites ubiquitin-binding protein p62/sequestosome 1 collagen XVII, FTLD-U/ALS, frontotemporal lobar degeneration with ubiquitinated inclusions and amyotrophic lateral sclerosis, IHC, immunohistochemistry Human Ubiquitin Nervous system diseases Glycoprotein Gene expression Binding protein Collagen Central nervous system disease Degenerative disease Spinal cord disease |
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Snippet | Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in locations in... Abstract Collagen involvement in motor neuron disease has been suggested by several earlier studies. Recently, we found collagen XVII to be expressed in... |
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SubjectTerms | Adaptor Proteins, Signal Transducing - genetics Adaptor Proteins, Signal Transducing - metabolism Adult Aged Aged, 80 and over Amyotrophic Lateral Sclerosis - genetics Amyotrophic Lateral Sclerosis - metabolism Amyotrophic Lateral Sclerosis - physiopathology Autoantigens - genetics Autoantigens - metabolism Biological and medical sciences Biomarkers - analysis Biomarkers - metabolism Brain - metabolism Brain - pathology Brain - physiopathology Cerebellum - metabolism Cerebellum - pathology Cerebellum - physiopathology Cerebrospinal fluid. Meninges. Spinal cord Collagen Type XVII Collagen XVII Comorbidity Cytoskeleton - metabolism Cytoskeleton - pathology Dermatologi och venerologi, klinisk genetik, invärtesmedicin Dermatology and venerology,clinical genetics, internal medicine Female Frontal Lobe - metabolism Frontal Lobe - pathology Frontal Lobe - physiopathology Hippocampus - metabolism Hippocampus - pathology Hippocampus - physiopathology Humans Immunohistochemistry Internal medicine Invärtesmedicin Male Medical sciences MEDICIN MEDICINE Middle Aged Morfologi, cellbiologi, patologi Morphology, cell biology, pathology Motor neuron disease Motor Neuron Disease - genetics Motor Neuron Disease - metabolism Motor Neuron Disease - physiopathology Nervous system (semeiology, syndromes) Neurocognitive Disorders - metabolism Neurocognitive Disorders - pathology Neurocognitive Disorders - physiopathology Neurologi Neurology Neurons - metabolism Neurons - pathology Non-Fibrillar Collagens - genetics Non-Fibrillar Collagens - metabolism p62 Pathology Patologi Sequestosome-1 Protein |
Title | Expression of collagen XVII and ubiquitin-binding protein p62 in motor neuron disease |
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