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 inBrain research Vol. 1247; pp. 171 - 177
Main Authors Seppänen, Allan, Pikkarainen, Maria, Hartikainen, Päivi, Hofmann, Silke C., Majamaa, Kari, Alafuzoff, Irina
Format Journal Article
LanguageEnglish
Published Amsterdam Elsevier B.V 09.01.2009
Elsevier
Subjects
p62
MND
NCI
PMA
NT
ALS
CNS
IR
p62
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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.
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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  givenname: Kari
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  email: irina.alafuzoff@uku.fi
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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
Language English
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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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https://dx.doi.org/10.1016/j.brainres.2008.10.020
https://www.ncbi.nlm.nih.gov/pubmed/18992722
https://www.proquest.com/docview/20246945
https://www.proquest.com/docview/66737215
https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-125862
Volume 1247
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