同: Hutchinson-Gil ford progeria syndrome

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1. 脂肪異栄養症症候群 lipodystrophic syndromes

English Journal

Sustained accumulation of prelamin a and depletion of lamin a/c both cause oxidative stress and mitochondrial dysfunction but induce different cell fates.

Sieprath T1, Corne TD, Nooteboom M, Grootaert C, Rajkovic A, Buysschaert B, Robijns J, Broers JL, Ramaekers FC, Koopman WJ, Willems PH, De Vos WH.
Nucleus (Austin, Tex.).Nucleus.2015 May 21:0. [Epub ahead of print]
The cell nucleus is structurally and functionally organized by lamins, intermediate filament proteins that form the nuclear lamina. Point mutations in genes that encode a specific subset of lamins, the A-type lamins, cause a spectrum of diseases termed laminopathies. Recent evidence points to a role
PMID 25996284

Satisfaction of health professionals after implementation of a primary care hospital emergency centre in Switzerland: A prospective before-after study.

Hess S1, Sidler P2, Chmiel C3, Bögli K2, Senn O4, Eichler K5.
International emergency nursing.Int Emerg Nurs.2015 May 4. pii: S1755-599X(15)00043-9. doi: 10.1016/j.ienj.2015.04.001. [Epub ahead of print]
BACKGROUND AND AIM: The increasing number of patients requiring emergency care is a challenge and leads to decreased satisfaction of health professionals at emergency departments (EDs). Thus, a Swiss hospital implemented a hospital-associated primary care centre at the ED. The study aim was to inves
PMID 25951785

MicroRNA transcriptome analysis identifies miR-365 as a novel negative regulator of cell proliferation in Zmpste24-deficient mouse embryonic fibroblasts.

Xiong XD1, Jung HJ2, Gombar S3, Park JY2, Zhang CL4, Zheng H4, Ruan J1, Li JB1, Kaeberlein M5, Kennedy BK6, Zhou Z7, Liu X8, Suh Y9.
Mutation research.Mutat Res.2015 Apr 24;777:69-78. doi: 10.1016/j.mrfmmm.2015.04.010. [Epub ahead of print]
Zmpste24 is a metalloproteinase responsible for the posttranslational processing and cleavage of prelamin A into mature laminA. Zmpste24-/- mice display a range of progeroid phenotypes overlapping with mice expressing progerin, an altered version of lamin A associated with Hutchinson-Gilford progeri
PMID 25983189

Japanese Journal

146 漢方療法を施行したハッチンソン・ギルフォード・プロジェリア症候群(HGPS)の1例(小児科疾患,一般演題,伝統医学のあるべきかたちとは-世界の潮流と日本の役割-,第59回日本東洋医学会学術総会)

山口英明
日本東洋醫學雜誌 59(別冊), 202, 2008-05-07
NAID 110006839463

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