全文获取类型
收费全文 | 151篇 |
免费 | 18篇 |
出版年
2023年 | 1篇 |
2022年 | 3篇 |
2021年 | 9篇 |
2020年 | 2篇 |
2019年 | 2篇 |
2018年 | 1篇 |
2017年 | 4篇 |
2016年 | 4篇 |
2015年 | 11篇 |
2014年 | 8篇 |
2013年 | 6篇 |
2012年 | 17篇 |
2011年 | 20篇 |
2010年 | 8篇 |
2009年 | 9篇 |
2008年 | 7篇 |
2007年 | 12篇 |
2006年 | 5篇 |
2005年 | 6篇 |
2004年 | 7篇 |
2003年 | 4篇 |
2002年 | 7篇 |
2000年 | 1篇 |
1997年 | 1篇 |
1996年 | 1篇 |
1995年 | 2篇 |
1992年 | 2篇 |
1990年 | 1篇 |
1987年 | 2篇 |
1986年 | 2篇 |
1981年 | 1篇 |
1976年 | 2篇 |
1975年 | 1篇 |
排序方式: 共有169条查询结果,搜索用时 22 毫秒
161.
Maryline Frelon-Raimond V. Benno Meyer-Rochow Alberto Ugolini Gilbert Martin 《Invertebrate Biology》2002,121(1):73-78
Abstract. No morphological clues on the amphipod head indicate the existence of ocelli. However, as in several isopod species studied so far, two rudimentary photoreceptors are integrated into the medio-dorsal part of the brain. This electron microscopical study of the photoreceptors is the first report on the presence of ocelli in amphipods. Each ocellus is made up of 3 receptor cells which contribute to the formation of a photoreceptive surface (the rhabdom) formed by tightly packed microvilli. The rhabdoms are twisted and irregular in outline. Membrane turnover is suggested by the presence of different kinds of lysosomes. Lacking dioptric lenses, these photoreceptors are not likely to be involved in image formation but may function as appraisers of ambient light intensity. Physiological and behavioral studies will, henceforth, have to take into account these unexpected ocelli, which may represent remnants of the naupliar eye. 相似文献
162.
163.
164.
Min-Wen Ku Maryline Bourgine Pierre Authié Jodie Lopez Kirill Nemirov Fanny Moncoq Amandine Noirat Benjamin Vesin Fabien Nevo Catherine Blanc Philippe Souque Houda Tabbal Emeline Simon David Hardy Marine Le Dudal Françoise Guinet Laurence Fiette Hugo Mouquet Pierre Charneau 《Cell host & microbe》2021,29(2):236-249.e6
165.
166.
167.
Anne Filipe Alexander Chernorudskiy Sandrine Arbogast Ersilia Varone Rocío-Nur Villar-Quiles Diego Pozzer Maryline Moulin Stefano Fumagalli Eva Cabet Swati Dudhal Maria-Grazia De Simoni Raphaël Denis Nathalie Vadrot Corinne Dill Matteo Giovarelli Luke Szweda Clara De Palma Paolo Pinton Carlotta Giorgi Carlo Viscomi Emilio Clementi Sonia Missiroli Simona Boncompagni Ester Zito Ana Ferreiro 《Cell death and differentiation》2021,28(1):123
SEPN1-related myopathy (SEPN1-RM) is a muscle disorder due to mutations of the SEPN1 gene, which is characterized by muscle weakness and fatigue leading to scoliosis and life-threatening respiratory failure. Core lesions, focal areas of mitochondria depletion in skeletal muscle fibers, are the most common histopathological lesion. SEPN1-RM underlying mechanisms and the precise role of SEPN1 in muscle remained incompletely understood, hindering the development of biomarkers and therapies for this untreatable disease. To investigate the pathophysiological pathways in SEPN1-RM, we performed metabolic studies, calcium and ATP measurements, super-resolution and electron microscopy on in vivo and in vitro models of SEPN1 deficiency as well as muscle biopsies from SEPN1-RM patients. Mouse models of SEPN1 deficiency showed marked alterations in mitochondrial physiology and energy metabolism, suggesting that SEPN1 controls mitochondrial bioenergetics. Moreover, we found that SEPN1 was enriched at the mitochondria-associated membranes (MAM), and was needed for calcium transients between ER and mitochondria, as well as for the integrity of ER-mitochondria contacts. Consistently, loss of SEPN1 in patients was associated with alterations in body composition which correlated with the severity of muscle weakness, and with impaired ER-mitochondria contacts and low ATP levels. Our results indicate a role of SEPN1 as a novel MAM protein involved in mitochondrial bioenergetics. They also identify a systemic bioenergetic component in SEPN1-RM and establish mitochondria as a novel therapeutic target. This role of SEPN1 contributes to explain the fatigue and core lesions in skeletal muscle as well as the body composition abnormalities identified as part of the SEPN1-RM phenotype. Finally, these results point out to an unrecognized interplay between mitochondrial bioenergetics and ER homeostasis in skeletal muscle. They could therefore pave the way to the identification of biomarkers and therapeutic drugs for SEPN1-RM and for other disorders in which muscle ER-mitochondria cross-talk are impaired.Subject terms: Chaperones, Respiratory tract diseases 相似文献
168.