全文获取类型
收费全文 | 1906篇 |
免费 | 136篇 |
出版年
2024年 | 2篇 |
2023年 | 15篇 |
2022年 | 42篇 |
2021年 | 100篇 |
2020年 | 49篇 |
2019年 | 65篇 |
2018年 | 75篇 |
2017年 | 55篇 |
2016年 | 81篇 |
2015年 | 133篇 |
2014年 | 143篇 |
2013年 | 167篇 |
2012年 | 199篇 |
2011年 | 170篇 |
2010年 | 101篇 |
2009年 | 85篇 |
2008年 | 109篇 |
2007年 | 85篇 |
2006年 | 72篇 |
2005年 | 60篇 |
2004年 | 63篇 |
2003年 | 44篇 |
2002年 | 29篇 |
2001年 | 10篇 |
2000年 | 6篇 |
1999年 | 2篇 |
1998年 | 5篇 |
1997年 | 5篇 |
1996年 | 5篇 |
1994年 | 2篇 |
1993年 | 3篇 |
1992年 | 6篇 |
1990年 | 5篇 |
1989年 | 4篇 |
1988年 | 4篇 |
1985年 | 11篇 |
1984年 | 4篇 |
1983年 | 2篇 |
1982年 | 1篇 |
1980年 | 3篇 |
1979年 | 1篇 |
1977年 | 2篇 |
1976年 | 5篇 |
1975年 | 2篇 |
1974年 | 3篇 |
1972年 | 1篇 |
1970年 | 2篇 |
1969年 | 1篇 |
1966年 | 1篇 |
1960年 | 1篇 |
排序方式: 共有2042条查询结果,搜索用时 312 毫秒
921.
Matteo Saladini Monica Nizzardo Alessandra Govoni Michela Taiana Nereo Bresolin Giacomo P. Comi Stefania Corti 《Journal of cellular and molecular medicine》2020,24(2):1169-1178
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive neuromuscular disorder caused by mutations in the IGHMBP2 gene, which encodes immunoglobulin μ‐binding protein 2, leading to progressive spinal motor neuron degeneration. We review the data available in the literature about SMARD1. The vast majority of patients show an onset of typical symptoms in the first year of life. The main clinical features are distal muscular atrophy and diaphragmatic palsy, for which permanent supportive ventilation is required. No effective treatment is available yet, but novel therapeutic approaches, such as gene therapy, have shown encouraging results in preclinical settings and thus represent possible methods for treating SMARD1. Significant advancements in the understanding of both the SMARD1 clinical spectrum and its molecular mechanisms have allowed the rapid translation of preclinical therapeutic strategies to human patients to improve the poor prognosis of this devastating disease. 相似文献
922.
Caroline F. Wright Nicholas M. Quaife Laura Ramos-Hernández Petr Danecek Matteo P. Ferla Kaitlin E. Samocha Joanna Kaplanis Eugene J. Gardner Ruth Y. Eberhardt Katherine R. Chao Konrad J. Karczewski Joannella Morales Giuseppe Gallone Meena Balasubramanian Siddharth Banka Lianne Gompertz Bronwyn Kerr Amelia Kirby Nicola Whiffin 《American journal of human genetics》2021,108(6):1083-1094
923.
Gregorio Oxilia Eugenio Bortolini Federica Badino Federico Bernardini Valentina Gazzoni Federico Lugli Matteo Romandini Anita Radini Gabriele Terlato Giulia Marciani Sara Silvestrini Jessica C. Menghi Sartorio Ursula Thun Hohenstein Luca Fiorenza Ottmar Kullmer Claudio Tuniz Jacopo Moggi Cecchi Sahra Talamo Federica Fontana Marco Peresani Stefano Benazzi Emanuela Cristiani 《American journal of physical anthropology》2021,174(2):232-253
924.
Gregorio Oxilia Jessica C. Menghi Sartorio Eugenio Bortolini Giulia Zampirolo Andrea Papini Marco Boggioni Sergio Martini Filippo Marciani Simona Arrighi Carla Figus Giulia Marciani Matteo Romandini Sara Silvestrini Maria Elena Pedrosi Tommaso Mori Alessandro Riga Ottmar Kullmer Rachel Sarig Luca Fiorenza Melchiore Giganti Rita Sorrentino Maria Giovanna Belcastro Jacopo Moggi Cecchi Stefano Benazzi 《American journal of physical anthropology》2021,175(4):847-864
925.
Nuclear molecules control the functional properties of the chromatin fiber by shaping its morphological properties. The biophysical mechanisms controlling how bridging molecules compactify chromatin are a matter of debate. On the one side, bridging molecules could cross-link faraway sites and fold the fiber through the formation of loops. Interacting bridging molecules could also mediate long-range attractions by first tagging different locations of the fiber and then undergoing microphase separation. Using a coarse-grained model and Monte Carlo simulations, we study the conditions leading to compact configurations both for interacting and noninteracting bridging molecules. In the second case, we report on an unfolding transition at high densities of the bridging molecules. We clarify how this transition, which disappears for interacting bridging molecules, is universal and controlled by entropic terms. In general, chains are more compact in the case of interacting bridging molecules because interactions are not valence limited. However, this result is conditional on the ability of our simulation methodology to relax the system toward its ground state. In particular, we clarify how, unless using reaction dynamics that change the length of a loop in a single step, the system is prone to remain trapped in metastable, compact configurations featuring long loops. 相似文献
926.
Marianna Carinci Beatrice Testa Matteo Bordi Giacomo Milletti Massimo Bonora Laura Antonucci Caterina Ferraina Marta Carro Mukesh Kumar Donatella Ceglie Franziska Eck Roberta Nardacci Francois le Guerrou Stefania Petrini Maria E Soriano Ignazio Caruana Valentina Doria Maria Manifava Camille Peron Matteo Lambrughi Valeria Tiranti Christian Behrends Elena Papaleo Paolo Pinton Carlotta Giorgi Nicholas T Ktistakis Franco Locatelli Francesca Nazio Francesco Cecconi 《The EMBO journal》2021,40(10)
The early secretory pathway and autophagy are two essential and evolutionarily conserved endomembrane processes that are finely interlinked. Although growing evidence suggests that intracellular trafficking is important for autophagosome biogenesis, the molecular regulatory network involved is still not fully defined. In this study, we demonstrate a crucial effect of the COPII vesicle‐related protein TFG (Trk‐fused gene) on ULK1 puncta number and localization during autophagy induction. This, in turn, affects formation of the isolation membrane, as well as the correct dynamics of association between LC3B and early ATG proteins, leading to the proper formation of both omegasomes and autophagosomes. Consistently, fibroblasts derived from a hereditary spastic paraparesis (HSP) patient carrying mutated TFG (R106C) show defects in both autophagy and ULK1 puncta accumulation. In addition, we demonstrate that TFG activity in autophagy depends on its interaction with the ATG8 protein LC3C through a canonical LIR motif, thereby favouring LC3C‐ULK1 binding. Altogether, our results uncover a link between TFG and autophagy and identify TFG as a molecular scaffold linking the early secretion pathway to autophagy. 相似文献
927.
Brenes-Peralta Laura Jiménez-Morales María Fernanda Campos-Rodríguez Rooel Vittuari Matteo 《The International Journal of Life Cycle Assessment》2021,26(10):2056-2071
The International Journal of Life Cycle Assessment - Several frameworks coincide in the importance of addressing social impacts to ensure sustainability. However, the agri-food sector, regarded as... 相似文献
928.
929.
930.
Umberto Maggi Dario Consonni Matteo Angelo Manini Stefano Gatti Francesco Cuccaro Francesca Donato Grazia Conte Pier Alberto Bertazzi Giorgio Rossi 《PloS one》2013,8(6)