Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1997-10-30
pubmed:abstractText
Myotonic dystrophy is an autosomal dominant multisystem disease primarily affecting skeletal muscle and is characterized by the presence of an amplified trinucleotide repeat in the 3' untranslated region of the myotonic dystrophy protein kinase gene. In this study, the subcellular localization of the myotonic dystrophy protein kinase in muscle tissues has been investigated at both morphological and biochemical level, by using antibodies against the myotonic dystrophy protein kinase. Immunofluorescence studies and Western-blot analysis were carried out with antibodies raised against both a synthetic peptide and a recombinant fusion protein fragment specific for the myotonic dystrophy protein kinase. The kinase is localized both to the surface membranes, and within the skeletal fibres in the region of the A-I band boundary. Consistent with the A-I location of the kinase is that Western-blot analysis of purified fractions from sarcoplasmic reticulum show that triads and sarcoplasmic reticulum terminal cisternae are immunoreactive for two myotonic dystrophy protein kinase proteins of different molecular weight (85 and 54 kDa). The relative amount of these two proteins is different in relation to the muscle type, the 85 kDa protein being more evident in skeletal than in cardiac fibres. In addition, immunofluorescence studies of cardiac muscle reveal a heavy concentration of DM-PK localized to the intercalated discs, as well as a weaker reaction in the sarcoplasm. These results taken together suggest that multiple isoforms of the DM-PK may exist and that they may be differentially located in muscle tissues.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0142-4319
pubmed:author
pubmed:issnType
Print
pubmed:volume
18
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
429-40
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1997
pubmed:articleTitle
Evidence for localization of the myotonic dystrophy protein kinase to the terminal cisternae of the sarcoplasmic reticulum.
pubmed:affiliation
Centro di Studio per la Biologia e la Fisiopatologia Muscolare del Consiglio Nazionale delle Ricerche, Dipartimento di Scienze Biomediche Sperimentali, University of Padova, Italy.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't