基本信息
views: 3
Career Trajectory
Bio
Research summary
Endocytic and phagocytic pathways introduce huge amounts of material into cells that are ultimately degraded within the lysosome. Cholesterol derived from internalized material, is transported from the lysosome to the ER. This delivery of cholesterol to the ER is a critical step in maintaining cholesterol homeostasis.
We have shown that the lysosome and the ER form transient connections where the membranes of the two organelles are physically associated. These connections, known as membrane contact sites, allow exchange of material and protein-protein interactions between the two organelles. We are trying to identify the proteins involved in regulating these contact sites and to understand the relationship between lipid transport and the ER-lysosome interface.
Defects in lipid transport result in lipid accumulation in lysosomes that is associated with several neurodegenerative diseases, including a rare but devastating childhood lysosomal storage disease, Niemann Pick type-C (NPC). Lipid accumulation is also believed to be a major contributing factor to the development of Age-related Macular Degeneration (AMD), the most common cause of vision loss in the elderly.
By learning more about the regulation of ER-lysosome contact sites and their role in lipid transport, we aim to identify tools with which to mobilise lipids from the lysosome to alleviate lysosomal storage defects in diseases such as NPC and AMD.
Endocytic and phagocytic pathways introduce huge amounts of material into cells that are ultimately degraded within the lysosome. Cholesterol derived from internalized material, is transported from the lysosome to the ER. This delivery of cholesterol to the ER is a critical step in maintaining cholesterol homeostasis.
We have shown that the lysosome and the ER form transient connections where the membranes of the two organelles are physically associated. These connections, known as membrane contact sites, allow exchange of material and protein-protein interactions between the two organelles. We are trying to identify the proteins involved in regulating these contact sites and to understand the relationship between lipid transport and the ER-lysosome interface.
Defects in lipid transport result in lipid accumulation in lysosomes that is associated with several neurodegenerative diseases, including a rare but devastating childhood lysosomal storage disease, Niemann Pick type-C (NPC). Lipid accumulation is also believed to be a major contributing factor to the development of Age-related Macular Degeneration (AMD), the most common cause of vision loss in the elderly.
By learning more about the regulation of ER-lysosome contact sites and their role in lipid transport, we aim to identify tools with which to mobilise lipids from the lysosome to alleviate lysosomal storage defects in diseases such as NPC and AMD.
Research Interests
Papers共 39 篇Author StatisticsCo-AuthorSimilar Experts
By YearBy Citation主题筛选期刊级别筛选合作者筛选合作机构筛选
时间
引用量
主题
期刊级别
合作者
合作机构
Proceedings of the National Academy of Sciences of the United States of Americano. 39 (2022): e2204396119-e2204396119
Duarte C Barral,Leopoldo Staiano,Cláudia Guimas Almeida, Dan F Cutler,Emily R Eden,Clare E Futter,Antony Galione,André R A Marques,Diego Luis Medina,Gennaro Napolitano,Carmine Settembre,Otília V Vieira,Johannes M F G Aerts,Peace Atakpa-Adaji,Gemma Bruno,Antonella Capuozzo,Elvira De Leonibus,Chiara Di Malta,Cristina Escrevente,Alessandra Esposito,Paolo Grumati,Michael J Hall,Rita O Teodoro,Susana S Lopes,J Paul Luzio,Jlenia Monfregola,Sandro Montefusco,Frances M Platt,Roman Polishchuck,Maria De Risi,Irene Sambri,Chiara Soldati,Miguel C Seabra
Frontiers in Cell and Developmental Biology (2022)
TRAFFICno. 5.0 (2020): 354.0-363
Load More
Author Statistics
#Papers: 39
#Citation: 3070
H-Index: 23
G-Index: 34
Sociability: 5
Diversity: 3
Activity: 21
Co-Author
Co-Institution
D-Core
- 合作者
- 学生
- 导师
Data Disclaimer
The page data are from open Internet sources, cooperative publishers and automatic analysis results through AI technology. We do not make any commitments and guarantees for the validity, accuracy, correctness, reliability, completeness and timeliness of the page data. If you have any questions, please contact us by email: report@aminer.cn