Results 81 to 90 of about 923,510 (301)

Diagnostic Utility of the ATG9A Ratio in AP‐4–Associated Hereditary Spastic Paraplegia

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Adaptor protein complex 4–associated hereditary spastic paraplegia (AP‐4‐HSP), a childhood‐onset neurogenetic disorder and frequent mimic of cerebral palsy, is caused by biallelic variants in the adaptor protein complex 4 (AP‐4) subunit genes (AP4B1 [for SPG47], AP4M1 [for SPG50], AP4E1 [for SPG51], and AP4S1 [for SPG52]).
Habibah A. P. Agianda, Hyo‐Min Kim, Nicole Battaglia, Joshua Rong, Amy Tam, Enrique Gonzalez Saez‐Diez, Cornelius F. Boerkoel, Afshin Saffari, Vicente Quiroz, Luca Schierbaum, Zainab Zaman, Katerina Bernardi, Darius Ebrahimi‐Fakhari   +12 more
wiley   +1 more source

In situ formed Mg(BH4)2 for improving hydrolysis properties of MgH2

Journal of Magnesium and Alloys
The hydrolysis of MgH2 delivers high hydrogen capacity (15.2 wt%), which is very attractive for real-time hydrogen supply. However, the formation of a surface passivation Mg(OH)2 layer and the large excess of H2O required to ensure complete hydrolysis ...
Yongyang Zhu, Mili Liu, liming Zeng, Yin Wang, Daifeng Wu, Rui Li, Qing Zhou, Renheng Tang, Fangming Xiao   +8 more
doaj   +1 more source

Excited bands in odd-mass rare-earth nuclei

, 2002
Normal parity bands are studied in 157Gd, 163Dy and 169Tm using the pseudo SU(3) shell model. Energies and B(E2) transition strengths of states belonging to six low-lying rotational bands with the same parity in each nuclei are presented. The pseudo SU(3)
Draayer, J. P., Hirsch, J. G., Vargas, C. E.   +2 more
core   +3 more sources

Clinical Spectrum and Outcomes of SOX1 Antibody‐Associated Paraneoplastic Neurological Syndromes: A Chinese Cohort Study

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background SOX1 antibody‐positive paraneoplastic neurological syndromes (PNS) exhibit significant population‐specific clinical heterogeneity. While Western cohorts predominantly manifest Lambert‐Eaton myasthenic syndrome (65%–80%), comprehensive clinical characterization and treatment response data in Asian populations remain critically ...
Jin‐Long Ye, Zu‐Ying Kuang, Bo Li, Meng‐Qiu Pan, Li‐Hua Zhou, Yang‐Yang Dai, Si‐Fen Xie, Xian‐Guang Lin, Ye‐Peng Hu, Li‐Hong Jiang, Zhan‐Hang Wang, Wei‐Jing Zhang   +11 more
wiley   +1 more source

Synthesis, Structure and Iodine Adsorption Properties of a Ni Cluster-Based Supramolecular Framework

Molecules
The capture of radioactive iodine (129I or 131I) is of significant importance for the production of nuclear power and the treatment of nuclear waste.
Jingyi Qiu, Linxia Tang, Ziang Nan, Luyao Liu, Qing Li, Wei Wang, Zhu Zhuo, Dongwei Zhang, Yougui Huang, Liangliang Zhang   +9 more
doaj   +1 more source

Persistent luminescence in rare-earth doped nitrido-silicates [PDF]

, 2011
Some luminescent materials are able to continue emitting light for hours after they have been excited, a phenomenon known as persistent luminescence. The majority of persistent phosphors known today are based on aluminates or silicates and emit in the ...
Poelman, Dirk, Smet, Philippe, Van den Eeckhout, Koen   +2 more
core  

Optical spectroscopy study on CeTe$_3$: evidence for multiple charge-density-wave orders

, 2010
We performed optical spectroscopy measurement on single crystal of CeTe$_3$, a rare-earth element tri-telluride charge density wave (CDW) compound. The optical spectra are found to display very strong temperature dependence.
Chen, Z. G., Cheng, B., Dong, T., Hu, B. F., Wang, N. L., Yuan, R. H., Zheng, P.   +6 more
core   +1 more source

Remote Assessment of Ataxia Severity in SCA3 Across Multiple Centers and Time Points

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Spinocerebellar ataxia type 3 (SCA3) is a genetically defined ataxia. The Scale for Assessment and Rating of Ataxia (SARA) is a clinician‐reported outcome that measures ataxia severity at a single time point. In its standard application, SARA fails to capture short‐term fluctuations, limiting its sensitivity in trials.
Marcus Grobe‐Einsler, Vivian Maas, Arian Taheri Amin, Jennifer Faber, Tamara Schaprian, Katharina Hill, Matthis Synofzik, Lisa H. Graf, Heike Jacobi, Manuela Lima, Ana F. Ferreira, Bart P. van de Warrenburg, Ilse H. J. Willemse, Dagmar Timmann, Andreas Thieme, Paola Giunti, Hector Garcia‐Moreno, Magda M. Santana, Jeannette Hübener‐Schmid, Elin H. Davies, Thomas Klockgether   +20 more
wiley   +1 more source

Coupling of a locally implanted rare-earth ion ensemble to a superconducting micro-resonator

, 2014
We demonstrate the coupling of rare-earth ions locally implanted in a substrate (Gd$^{3+}$ in Al$_{2}$O$_{3}$) to a superconducting NbN lumped-element micro-resonator. The hybrid device is fabricated by a controlled ion implantation of rare-earth ions in
Adamyan, A., de Graaf, S. E., Gwilliam, R., Kubatkin, S., Lindström, T., Meeson, P. J., Tzalenchuk, A. Ya., Wisby, I.   +7 more
core   +1 more source

Diffusion Spectrum Imaging Maps Early Axonal Loss and a Unique Progressive Signal in Neuronal Intranuclear Inclusion Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To delineate specific in vivo white matter pathology in neuronal intranuclear inclusion disease (NIID) using diffusion spectrum imaging (DSI) and define its clinical relevance. Methods DSI was performed on 42 NIID patients and 38 matched controls.
Kaiyan Jiang, Yixiu Pei, Xiaobao Hu, Ying Xiong, Fang Wang, Yanyan Yu, Min Zhu, Dandan Tan, Meihong Zhou, Daojun Hong, Fuqing Zhou   +10 more
wiley   +1 more source