Article
Biochemistry & Molecular Biology
Nuomin Li, Yunjie Qiu, Hao Wang, Juan Zhao, Hong Qing
Summary: Neuritic plaques, major pathological features of Alzheimer's disease, are formed by the aggregation of A beta protein derived from APP cleavage by BACE1 and gamma-secretase, with mutations in PSEN1 gene affecting the activity of gamma-secretase and contributing to early onset familial Alzheimer's disease.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Yong Bhum Song, Seung-Yeol Park, Kunyou Park, Hayoung Hwang, Rona S. Carroll, Victor W. Hsu, Ursula B. Kaiser
Summary: G protein-coupled receptors (GPCRs) play crucial roles in physiological and pathological processes, and mutations can lead to diseases. This study focuses on PROKR2 and its mutant causing hypogonadotropic hypogonadism, and identifies distinct interactomes and post-ER itinerary that determine the fate of the wild-type and mutant receptors. The study also reveals the role of the post-ER itinerary in reducing ER stress induced by mutant PROKR2.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Review
Cell Biology
Aurel George Mohan, Bogdan Calenic, Nicu Adrian Ghiurau, Roxana-Maria Duncea-Borca, Alexandra-Elena Constantinescu, Ileana Constantinescu
Summary: This comprehensive review article delves into the Golgi apparatus, a crucial organelle in cellular biology. It discusses its unique organization and roles in maintaining cellular homeostasis through protein processing, sorting, and lipid biogenesis. The article also explores the relationship between Golgi function and neurodegenerative diseases, particularly Parkinson's, as well as the interplay between Golgi stress and endoplasmic reticulum stress in cellular stress response pathways. Overall, the review emphasizes the importance of further research in understanding Golgi dysfunction and its potential for targeted therapeutic approaches.
Article
Cell Biology
Elena Morelli, Elisa A. Speranza, Enrica Pellegrino, Galina Beznoussenko, Francesca Carminati, Massimiliano Garre, Alexander A. Mironov, Marco Onorati, Thomas Vaccari
Summary: Snap29 is a crucial regulator of membrane fusion, essential for cellular processes such as autophagy and cell division. Mutations in Snap29 can lead to alterations in Golgi apparatus and disrupted ER to GA trafficking, revealing a new regulatory function of Snap29 in promoting secretory trafficking.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Patricia Franzka, Svenja Caren Schueler, Takfarinas Kentache, Robert Storm, Andrea Bock, Istvan Katona, Joachim Weis, Katrin Buder, Christoph Kaether, Christian A. Hubner
Summary: Proteins of the secretory pathway undergo glycosylation in the ER and Golgi apparatus. Mutations in GMPPA can cause AAMR syndrome with various symptoms. Loss of GMPPA leads to Golgi fragmentation, altered protein abundance, reduced furin activity, and increased retention of alpha-DG.
Article
Biochemistry & Molecular Biology
Arisa Ochiai, Sui Sawaguchi, Shiori Memezawa, Yoichi Seki, Takako Morimoto, Hiroaki Oizumi, Katsuya Ohbuchi, Masahiro Yamamoto, Kazushige Mizoguchi, Yuki Miyamoto, Junji Yamauchi
Summary: The study demonstrated that the AIMP2 mutant proteins associated with HLD17 localize in the Golgi bodies, stimulating Golgi stress signaling and inhibiting differentiation. Knockdown of CASP2 reversed the inhibitory effect, suggesting a potential approach to ameliorating the disease's effects.
NEUROCHEMICAL RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Alicja Koscielny, Ewa Liszewska, Katarzyna Machnicka, Michalina Wezyk, Katarzyna Kotulska, Jacek Jaworski
Summary: mTOR inhibition enhances cargo transport through the secretory pathway, particularly in PC12 cells and primary human fibroblasts. Additionally, VSVg trafficking is increased in TSC1-deficient cells.
CELLULAR & MOLECULAR BIOLOGY LETTERS
(2021)
Article
Neurosciences
Ruoyu Duan, Liuju Li, Huifang Yan, Miao He, Kai Gao, Shijia Xing, Haoran Ji, Jianyong Wang, Binbin Cao, Dongxiao Li, Han Xie, Shiqun Zhao, Ye Wu, Yuwu Jiang, Jiangxi Xiao, Qiang Gu, Ming Li, Xiaolu Zheng, Liangyi Chen, Jingmin Wang
Summary: This study elucidated the relationship between PLP1 duplication and mitochondrial dysfunction, demonstrating closer ER-mitochondrion interfaces and structural changes in ER and MAMs. These findings provide novel insights into the pathogenic mechanisms of hypomyelinating leukodystrophies diseases at the organelle level.
Review
Virology
Kaitlyn Speckhart, Jeffrey M. Williams, Billy Tsai
Summary: Viruses use host molecular chaperones to enter, replicate, and assemble within host cells. DNA viruses like polyomavirus and human papillomavirus utilize soluble and membrane-associated chaperones, while RNA viruses like flavivirus and coronavirus co-opt cytosolic and organelle-selective chaperones. This highlights the significance of host chaperones in virus infection and potential for novel antiviral strategies.
Review
Biochemistry & Molecular Biology
Lana Buzuk, Doris Hellerschmied
Summary: The Golgi apparatus plays a crucial role in the secretory pathway of eukaryotic cells, processing proteins and organizing their transportation to other cellular compartments. It is involved in cell surface formation, cell polarity, cell-cell communication, immune signaling, DNA damage response, and mitosis. Maintaining Golgi integrity and protein homeostasis is essential, as Golgi fragmentation and dysfunction are linked to neurodegenerative diseases and certain cancers. Recent studies have revealed the importance of ubiquitin signaling in maintaining Golgi integrity and protein quality control, similar to the endoplasmic reticulum. Ubiquitination helps prevent the accumulation of toxic protein aggregates and regulates Golgi structural rearrangements in response to cellular stress.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Anja Konietzny, Jasper Grendel, Alan Kadek, Michael Bucher, Yuhao Han, Nathalie Hertrich, Dick H. W. Dekkers, Jeroen A. A. Demmers, Kay Gruenewald, Charlotte Uetrecht, Marina Mikhaylova
Summary: Excitatory synapses of principal hippocampal neurons are often found on dendritic spines, with dynamic strengthening or weakening of individual inputs leading to structural and molecular diversity of spines. Caldendrin, a Ca2+ sensor, regulates the localization of ER tubules and formation of the spine apparatus in dendritic spines through a myosin V-dependent pathway, transforming myosin into a stationary F-actin tether.
Article
Clinical Neurology
Anna Skupien-Jaroszek, Andrzej A. Szczepankiewicz, Andrzej Rysz, Andrzej Marchel, Ewa Matyja, Wieslawa Grajkowska, Grzegorz M. Wilczynski, Joanna Dzwonek
Summary: This study examined the changes in Golgi apparatus (GA) architecture in epilepsy. The researchers observed GA dispersion in neurons of epilepsy patients and rats with induced seizures. They found that the structural changes of GA were reversible and that enhanced neuronal activity played a role in the Golgi reorganization. The study also revealed the importance of elevated cytosolic Ca2+ in the KA-induced morphological alterations of GA in vitro.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)
Article
Clinical Neurology
Anna Skupien-Jaroszek, Andrzej A. Szczepankiewicz, Andrzej Rysz, Andrzej Marchel, Ewa Matyja, Wieslawa Grajkowska, Grzegorz M. Wilczynski, Joanna Dzwonek
Summary: In this study, we have observed the dispersion of the neuronal Golgi apparatus (GA) in the human brain of patients with epilepsy and rat brain upon seizures. The changes in GA structure were reversible and could be prevented by BAPTA-AM, suggesting that they are induced by elevated cytosolic Ca2+. These findings indicate that GA fragmentation is involved in aberrant neuronal plasticity processes underlying epilepsy.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)
Review
Clinical Neurology
Maryline Santerre, Sterling P. Arjona, Charles N. S. Allen, Natalia Shcherbik, Bassel E. Sawaya
Summary: The NSPs of the virus play a crucial role in establishing the replication and transcription complex by hijacking the endoplasmic reticulum membrane, potentially leading to central nervous system damage and neurodegeneration. Research on these NSPs will not only reveal their specific role in viral infection but also aid in the discovery of novel targeted drugs.
JOURNAL OF NEUROLOGY
(2021)
Review
Cell Biology
Amrita Kulkarni, Kumari Preeti, Kamatham Pushpa Tryphena, Saurabh Srivastava, Shashi Bala Singh, Dharmendra Kumar Khatri
Summary: Protein dyshomeostasis plays a key role in age-related neurodegenerative disorders, such as Parkinson's disease, characterized by the deposition of Lewy bodies composed of alpha-synuclein protein aggregates. The proper functioning of proteostasis, including unfolded protein response, ubiquitin-proteasome system, and autophagy-lysosome pathway, is crucial for protein synthesis, folding, confirmation, and degradation. Mutational changes in the genes involved in these pathways, as well as epigenetic modifications and microRNA-mediated transcriptional changes, contribute to proteostasis dysregulation. Defective proteostasis leads to the deposition of proteins, which can serve as potential biomarkers for early diagnosis of Parkinson's disease. Targeting different aspects of proteostasis machinery holds promise for delaying the accumulation of pathological hallmarks.
AGEING RESEARCH REVIEWS
(2023)