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rdf:type |
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lifeskim:mentions |
umls-concept:C0001898,
umls-concept:C0016315,
umls-concept:C0024485,
umls-concept:C0041249,
umls-concept:C0150369,
umls-concept:C0205341,
umls-concept:C0206243,
umls-concept:C1418237,
umls-concept:C1514562,
umls-concept:C1522492,
umls-concept:C1880389,
umls-concept:C1883204,
umls-concept:C1883221,
umls-concept:C1948066
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pubmed:issue |
11
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pubmed:dateCreated |
2008-5-9
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|
pubmed:abstractText |
Intranuclear fibrils due to poly-alanine expansions in the N-terminal domain of the poly(A) binding protein PABPN1 correlate with the disease oculopharyngeal muscular dystrophy (OPMD). For monitoring fibril formation by fluorescence and real-time NMR spectroscopy, tryptophans were introduced either into the middle or C-terminal of the poly-alanine segment. The kinetics of fibril formation which were monitored by fluorescence spectroscopy were matched by real-time NMR kinetics. Our results show that fibril formation is concomitant with the burial of the tryptophans in the fibrillar core. Since no soluble pre-fibrillar intermediate(s) was detected, fibril formation of this domain may be regarded as a two state conversion from an unfolded soluble into folded insoluble species.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
0014-5793
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pubmed:author |
|
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pubmed:issnType |
Print
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pubmed:day |
14
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pubmed:volume |
582
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1587-92
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pubmed:meshHeading |
pubmed-meshheading:18406354-Alanine,
pubmed-meshheading:18406354-Amino Acid Sequence,
pubmed-meshheading:18406354-Amyloid,
pubmed-meshheading:18406354-Fluorescence,
pubmed-meshheading:18406354-Humans,
pubmed-meshheading:18406354-Molecular Sequence Data,
pubmed-meshheading:18406354-Nuclear Magnetic Resonance, Biomolecular,
pubmed-meshheading:18406354-Poly(A)-Binding Protein II,
pubmed-meshheading:18406354-Protein Structure, Tertiary,
pubmed-meshheading:18406354-Recombinant Proteins,
pubmed-meshheading:18406354-Repetitive Sequences, Amino Acid,
pubmed-meshheading:18406354-Tryptophan
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pubmed:year |
2008
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pubmed:articleTitle |
Monitoring fibril formation of the N-terminal domain of PABPN1 carrying an alanine repeat by tryptophan fluorescence and real-time NMR.
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pubmed:affiliation |
Institute for Biochemistry and Biotechnology of the Martin-Luther-University Halle-Wittenberg, Kurt-Mothes-Strasse 3, 06120 Halle, Germany.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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