| Item type |
学術雑誌論文 / Journal Article(1) |
| 公開日 |
2010-08-19 |
| タイトル |
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|
タイトル |
＜Poster＞Pyroglutamate formation at the N-termini of ABri molecules in familial British dementia is not restricted to the central nervous system |
| 言語 |
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|
言語 |
eng |
| キーワード |
|
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主題Scheme |
Other |
|
主題 |
familial British dementia |
| キーワード |
|
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主題Scheme |
Other |
|
主題 |
ABri |
| キーワード |
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主題Scheme |
Other |
|
主題 |
pyroglutamate |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
amyloid |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
post-translational modifi cation |
| 資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| 著者 |
Tomidokoro, Yasushi
Tamaoka, Akira
Holton, Janice L.
Lashley, Tammaryn
Frangione, Blas
Revesz, Tamas
Rostagno, Agueda
Ghiso, Jorge
|
| 著者所属 |
|
|
値 |
Department of Neurology, University of Tsukuba, Graduate School of Comprehensive Human Science |
| 著者所属 |
|
|
値 |
Department of Neurology, University of Tsukuba, Graduate School of Comprehensive Human Science |
| 著者所属 |
|
|
値 |
UCL Institute of Neurology, University College London |
| 著者所属 |
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|
値 |
UCL Institute of Neurology, University College London |
| 著者所属 |
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|
値 |
Department of Pathology, New York University, School of Medicine |
| 著者所属 |
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値 |
UCL Institute of Neurology, University College London |
| 著者所属 |
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値 |
Department of Pathology, New York University, School of Medicine |
| 著者所属 |
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|
値 |
Department of Pathology, New York University, School of Medicine |
| 抄録 |
|
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内容記述タイプ |
Abstract |
|
内容記述 |
Amyloid molecules harboring pyroglutamate （pGlu） residue at the N-termini are considered to be important for the development of cerebral amyloidosis such as Alzheimer’s disease and thought to be either spontaneously generated or being catalyzed by glutaminyl cyclase. Familial British dementia （FBD） is an autosomal dominant form of dementia neuropathologically characterized by parenchymal amyloid and preamyloid deposits, extensive cerebral amyloid angiopathy, and neurofi brillary tangles. FBD is caused by a stop to Arg mutation in the BRI2 gene, generating de novo created amyloid molecule ABri which accumulates in FBD brains but is not present in the normal population. Soluble ABri molecules present in the circulation of carriers of the BRI2 mutation are 34 amino acids long exclusively harboring Glu residue at the N-termini （ABri1-34E）, whereas water- and formic acidsoluble ABri molecules extracted from FBD brains have abundant ABri species bearing pGlu residue （ABri1-34pE）, suggesting that pyroglutamate formation occurs at the site of deposition. In order to further clarify the mechanism（s） of ABri deposition, we studied whether pyroglutamate formation indeed occurs outside the central nervous system taking advantage that FBD is also a systemic amyloidosis. Soluble and fibrillar ABri molecules extracted from systemic organs and analyzed biochemically using a combination of immunoprecipitation, mass spectrometry, and western blot analysis were oligomeric in size and contained a large proportion of ABri1-34pE. The data indicate that pyroglutamate formation at the N-termini of ABri molecules is an early step in the process of FBD amyloid deposition, and its formation is not restricted to the central nervous system. |
| 引用 |
|
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内容記述タイプ |
Other |
|
内容記述 |
弘前医学. 61(Suppl.), 2010, p.S262-S269 |
| 書誌情報 |
弘前医学
巻 61,
号 Supplement,
p. S262-S269,
発行日 2010-07-08
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| ISSN |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
0439-1721 |
| 書誌レコードID |
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収録物識別子タイプ |
NCID |
|
収録物識別子 |
AN00211444 |
| 著者版フラグ |
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出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| 日本十進分類法 |
|
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主題Scheme |
NDC |
|
主題 |
490 |
| NIIサブジェクト |
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主題Scheme |
Other |
|
主題 |
医学 |
| 出版者 |
|
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出版者 |
弘前大学大学院医学研究科・弘前医学会 |
| 資源タイプ |
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|
値 |
Article |
HirosakiMedJ_61_S262.pdf (239.4 kB)
