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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
1991-4-9
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pubmed:abstractText |
Our previous study disclosed strong immunostaining of brain tissue in neuronal ceroid lipofuscinosis (NCL) with antibodies against amyloid beta-protein and the presence of 31-kDa polypeptide in the storage material. In the present study, we investigated the immunoreactivity of the NCL brain tissue with anti-serum (anti-GID) raised against a synthetic peptide, based on the amyloid beta-protein precursor, with the 175-186 amino acid sequence. Immunocytochemistry was performed on autopsy brain material collected from 15 NCL cases, and from 8 age-matched normal controls. The results showed strong immunoreactivity of nerve cells in the NCL cases, which according to densitometry was 5 times more intense than in the control group (P less than 0.0001 by Student's t-test). Western blot analysis revealed that in protein fractions of NCL brain homogenates anti-GID recognized the protein band of 35 kDa. Thus our present and previously performed studies supplied for the first time data pointing to abnormal processing of amyloid beta-protein precursor in NCL. Moreover, the accumulation of both 31- and 35-kDa polypeptides that was demonstrated provides further support for postulated defective protein metabolism in this disorder.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0304-3940
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
27
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pubmed:volume |
120
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
94-6
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pubmed:dateRevised |
2010-11-18
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pubmed:meshHeading |
pubmed-meshheading:2127306-Amyloid beta-Peptides,
pubmed-meshheading:2127306-Amyloid beta-Protein Precursor,
pubmed-meshheading:2127306-Autopsy,
pubmed-meshheading:2127306-Axons,
pubmed-meshheading:2127306-Brain,
pubmed-meshheading:2127306-Cerebral Cortex,
pubmed-meshheading:2127306-Humans,
pubmed-meshheading:2127306-Immunoenzyme Techniques,
pubmed-meshheading:2127306-Neuronal Ceroid-Lipofuscinoses,
pubmed-meshheading:2127306-Neurons,
pubmed-meshheading:2127306-Protease Inhibitors,
pubmed-meshheading:2127306-Protein Precursors,
pubmed-meshheading:2127306-Reference Values
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pubmed:year |
1990
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pubmed:articleTitle |
Altered amyloid beta-protein precursor processing in brains of patients with neuronal ceroid lipofuscinosis.
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pubmed:affiliation |
New York State Office of Mental Retardation and Developmental Disabilities, Department of Pathological Neurobiology, Staten Island 10314.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.
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