Expression of collagen XVII and ubiquitin-binding protein p62 in motor neuron disease

• Published in: Brain research Vol. 1247; pp. 171 - 177
• Main Authors: Seppänen, Allan, Pikkarainen, Maria, Hartikainen, Päivi, Hofmann, Silke C., Majamaa, Kari, Alafuzoff, Irina
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 09.01.2009; Elsevier
• Subjects: 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; 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

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...