التفاصيل البيبلوغرافية
| العنوان: |
Dysfunction of RAB39B-Mediated Vesicular Trafficking in Lewy Body Diseases |
| المؤلفون: |
Flaviano Giorgini, Tiago F. Outeiro, Susanna Campesan, David J. Koss |
| المصدر: |
Movement disorders 36(8), 1744-1758 (2021). doi:10.1002/mds.28605 |
| بيانات النشر: |
Wiley, 2021. |
| سنة النشر: |
2021 |
| مصطلحات موضوعية: |
0301 basic medicine, Lewy Body Disease, Neurite, Endosome, alpha-synuclein, GTPase, Biology, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, genetics [Lewy Body Disease], genetics [Parkinson Disease], medicine, Humans, endocytosis, ddc:610, metabolism [alpha-Synuclein], Alpha-synuclein, Lewy body, RAB39B, Autophagy, Neurodegeneration, neurodegeneration, Parkinson Disease, Lewy body diseases, medicine.disease, metabolism [Lewy Bodies], metabolism [rab GTP-Binding Proteins], 030104 developmental biology, Neurology, chemistry, rab GTP-Binding Proteins, alpha-Synuclein, genetics [alpha-Synuclein], Lewy Bodies, Neurology (clinical), Rab, genetics [rab GTP-Binding Proteins], Neuroscience, 030217 neurology & neurosurgery |
| الوصف: |
Intracellular vesicular trafficking is essential for neuronal development, function, and homeostasis and serves to process, direct, and sort proteins, lipids, and other cargo throughout the cell. This intricate system of membrane trafficking between different compartments is tightly orchestrated by Ras analog in brain (RAB) GTPases and their effectors. Of the 66 members of the RAB family in humans, many have been implicated in neurodegenerative diseases and impairment of their functions contributes to cellular stress, protein aggregation, and death. Critically, RAB39B loss-of-function mutations are known to be associated with X-linked intellectual disability and with rare early-onset Parkinson's disease. Moreover, recent studies have highlighted altered RAB39B expression in idiopathic cases of several Lewy body diseases (LBDs). This review contextualizes the role of RAB proteins in LBDs and highlights the consequences of RAB39B impairment in terms of endosomal trafficking, neurite outgrowth, synaptic maturation, autophagy, as well as alpha-synuclein homeostasis. Additionally, the potential for therapeutic intervention is examined via a discussion of the recent progress towards the development of specific RAB modulators. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. |
| اللغة: |
English |
| الوصول الحر: |
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::174a93c41786ddafd79e4ed29cf4a7d4Test
https://pub.dzne.de/record/155579Test |
| حقوق: |
OPEN |
| رقم الانضمام: |
edsair.doi.dedup.....174a93c41786ddafd79e4ed29cf4a7d4 |
| قاعدة البيانات: |
OpenAIRE |
