Serendipitous effects of β-cyclodextrin on murine model of Krabbe disease.
Serendipitous effects of β-cyclodextrin on murine model of Krabbe disease.
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DOI：
10.1016/j.ymgmr.2018.03.002
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发表时间：
2018-06
影响因子：
1.9
通讯作者：
Maegawa GHB
中科院分区：
文献类型：
--
作者：
Katabuchi AU;Godoy V;Shil P;Moser A;Maegawa GHB
图1所示。2-羟丙基-β-环糊精（HPβCD）对克拉伯病Twitcher小鼠模型的影响。(A) HPβCD治疗Twitcher （Twi galctwi/ Twi）小鼠（n= 34）和对照组（n= 51）的生存分析。两组均采用相同数量的双母、双母、双母。Twi组小鼠从出生后第3天起至死亡，每2天皮下注射4500mg /kg磷酸盐稀释的HPβCD。初始组12只小鼠也以相同的频率和持续时间皮下注射3500mg /kg，纳入生存分析。LC-MS/MS测定的脑(B)和坐骨神经(C)样品中精神碱水平在接受hc - β cd治疗的小鼠（n= 6）和只接受生理盐水治疗的小鼠（n= 6）之间无统计学差异。(D)接受hc - β cd治疗的小鼠（n= 6）髓鞘轴突球数较对照组（n= 6）显著增加（p< 0.05）。(E)死后解剖的坐骨神经的透射电镜图像显示，在接受HPβCD的Twi小鼠组中，髓鞘纤维的保存增加。https://doi。org/10.1016/j。ymgmr。2018.03. 2018年3月2日收到；2018年3月2日收到修改后的表格；2018年3月3日接受
Fig. 1. The effects of 2-hydroxypropyl-β-cyclodextrin (HPβCD) in the Twitcher mouse model for Krabbe disease.(A) Survival analysis of Twitcher (Twi galctwi/twi) mice treated with HPβCD (n= 34) and controls (n= 51). Equal number of males and females of Twi galctwi/twi were used in both groups. From post-natal day 3 until their death, treated Twi mice group received 4500 mg/kg of HPβCD subcutaneously diluted in phosphate saline every 2 days. An initial group of 12 mice receiving 3500 mg/kg subcutaneously also in the same frequency and duration are included in this survival analysis. Psychosine levels measured by LC-MS/MS in the brain (B) and sciatic nerve (C) specimens showed no statistically significant differences between mice receiving HCβCD (n= 6) and those receiving only saline (n= 6).(D) The number of myelinated axonal bulbs were significantly increased in the mice receiving HCβCD (n= 6) in comparison to controls (n= 6)(p< 0.05).(E) The transmission electron microscopy images of sciatic nerves dissected post-mortem showed increased preservation of myelinated fibers in the group of Twi mice receiving HPβCD. https://doi. org/10.1016/j. ymgmr. 2018.03. 002 Received 2 March 2018; Received in revised form 2 March 2018; Accepted 3 March 2018
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DOI：
10.1016/s0140-6736(17)31465-4
发表时间：
2017-10-14
期刊：
Lancet (London, England)
影响因子：
--
作者：
Ory DS;Ottinger EA;Farhat NY;King KA;Jiang X;Weissfeld L;Berry-Kravis E;Davidson CD;Bianconi S;Keener LA;Rao R;Soldatos A;Sidhu R;Walters KA;Xu X;Thurm A;Solomon B;Pavan WJ;Machielse BN;Kao M;Silber SA;McKew JC;Brewer CC;Vite CH;Walkley SU;Austin CP;Porter FD
通讯作者：
Porter FD
影响因子：
3.8
作者：
Ribbens, Jameson. J.;Moser, Ann B.;Maegawa, Gustavo H. B.
通讯作者：
Maegawa, Gustavo H. B.
影响因子：
1.9
作者：
Suzuki, K
通讯作者：
Suzuki, K
影响因子：
3.7
作者：
Yergey, Alfred L.;Blank, Paul S.;Darling, Allan J.
通讯作者：
Darling, Allan J.
DOI：
10.1523/jneurosci.1317-11.2011
发表时间：
2011-06-22
期刊：
The Journal of neuroscience : the official journal of the Society for Neuroscience
影响因子：
--
作者：
Aqul A;Liu B;Ramirez CM;Pieper AA;Estill SJ;Burns DK;Liu B;Repa JJ;Turley SD;Dietschy JM
通讯作者：
Dietschy JM