Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2002-5-1
pubmed:abstractText
Inclusion body myositis (IBM), the most common age-related muscle disease in the elderly population, is an incurable disorder leading to severe disability. Sporadic IBM has an unknown etiology, although affected muscle fibers are characterized by many of the pathobiochemical alterations traditionally associated with neurodegenerative brain disorders such as Alzheimer's disease. Accumulation of the amyloid-beta peptide, which is derived from proteolysis of the larger amyloid-beta precursor protein (betaAPP), seems to be an early pathological event in Alzheimer's disease and also in IBM, where in the latter, it predominantly occurs intracellularly within affected myofibers. To elucidate the possible role of betaAPP mismetabolism in the pathogenesis of IBM, transgenic mice were derived in which we selectively targeted betaAPP overexpression to skeletal muscle by using the muscle creatine kinase promoter. Here we report that older (>10 months) transgenic mice exhibit intracellular immunoreactivity to betaAPP and its proteolytic derivatives in skeletal muscle. In this transgenic model, selective overexpression of betaAPP leads to the development of a subset of other histopathological and clinical features characteristic of IBM, including centric nuclei, inflammation, and deficiencies in motor performance. These results are consistent with a pathogenic role for betaAPP mismetabolism in human IBM.
pubmed:grant
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10207012, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10400697, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10487842, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10590889, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10623648, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10702395, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10722712, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10816583, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-10901230, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-11035007, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-11211236, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-11242064, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-11274343, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-1321564, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-1423335, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-1668275, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-2440339, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-2796990, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-4384609, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-5095321, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-7692809, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-7704018, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-7746481, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-7905181, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8117535, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8191290, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8280854, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8291607, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8394158, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8577761, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8626743, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8705854, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8756664, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8773613, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-8896570, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9108116, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9218507, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9227956, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9243602, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9546349, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9585420, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9812213, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9846957, http://linkedlifedata.com/resource/pubmed/commentcorrection/11972038-9846958
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0027-8424
pubmed:author
pubmed:issnType
Print
pubmed:day
30
pubmed:volume
99
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
6334-9
pubmed:dateRevised
2009-11-18
pubmed:meshHeading
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