Article
Multidisciplinary Sciences
Navit Ogen-Shtern, Chieh Chang, Haddas Saad, Niv Mazkereth, Chaitanya Patel, Marina Shenkman, Gerardo Z. Lederkremer
Summary: Misfolded proteins and components of the ER quality control and ERAD machineries accumulate in the pericentriolar ERQC, which serves as a staging ground for ERAD. Trafficking to the ERQC is reversible, with recycling back to the ER slower than movement in the ER periphery. Vesicular trafficking, rather than diffusion, is involved in this process. COPI inhibition leads to accumulation in the ERQC and increases ERAD, while COPII inhibition has the opposite effect. Targeting of misfolded proteins to ERAD involves COPII-dependent transport to the ERQC and retrieval to the peripheral ER in a COPI-dependent manner.
Review
Biochemistry & Molecular Biology
Keisuke Mochida, Hitoshi Nakatogawa
Summary: Autophagy is a degradation system in eukaryotic cells that controls the mass and functions of organelles by degrading excess or defective portions. The endoplasmic reticulum (ER) is an organelle with specific functions that dynamically changes its mass and shape to maintain or regulate its functions. Elaborate mechanisms are required for the proper degradation of the ER.
Article
Biochemistry & Molecular Biology
Kohta Miura, Riko Katsuki, Shusei Yoshida, Ren Ohta, Taku Tamura
Summary: ERAD is an essential process for maintaining cell function and preventing ER stress. EDEM1 was found to be involved in accelerating the degradation of EGFR via ERAD, highlighting its physiological role in maintaining specific target proteins and providing a potential approach to regulate the expression of clinically important proteins.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Physiology
Fulvio Reggiori, Maurizio Molinari
Summary: ER-phagy is the process of degrading portions of the endoplasmic reticulum (ER) within lysosomes or vacuoles. It plays a role in recycling cytoplasmic material and organelles, regulating ER size and activity, and removing potentially cytotoxic material. Dysfunctional ER-phagy is associated with specific human diseases and can be targeted by pathogens.
PHYSIOLOGICAL REVIEWS
(2022)
Article
Multidisciplinary Sciences
Gunes Parlakgul, Ana Paula Arruda, Song Pang, Erika Cagampan, Nina Min, Ekin Guney, Grace Yankun Lee, Karen Inouye, Harald F. Hess, C. Shan Xu, Gokhan S. Hotamisligil
Summary: This study reveals the complex structural organization of organelles in liver tissue and highlights the significant alterations in obese mice. The functional importance of these structural changes on cellular and systemic metabolism is also demonstrated.
Article
Cell Biology
Jacob A. Culver, Malaiyalam Mariappan
Summary: The study found that tail-anchored proteins are recognized and ubiquitinated by cytosolic protein quality control system upon synthesis, but are not degraded by proteasomes. Instead, they are delivered to the ER for insertion, where deubiquitinases USP20 and USP33 remove ubiquitin chains.
JOURNAL OF CELL BIOLOGY
(2021)
Review
Biochemistry & Molecular Biology
John C. Christianson, Pedro Carvalho
Summary: This article discusses recent advances in understanding the mechanisms of ERAD and its impact on the regulation of ER functions.
Article
Plant Sciences
Xifeng Li, Zhe Wang, Yunting Fu, Xi Cheng, Yan Zhang, Baofang Fan, Cheng Zhu, Zhixiang Chen
Summary: UBAC2 is a multifunctional protein in plant cells that regulates the degradation and accumulation of specific proteins, including COPT and POWDERY MILDEW RESISTANCE 4.
Article
Biochemistry & Molecular Biology
Alicia Otero, Marina Betancor, Hasier Erana, Natalia Fernandez Borges, Jose J. Lucas, Juan Jose Badiola, Joaquin Castilla, Rosa Bolea
Summary: Prion diseases are a group of neurodegenerative disorders caused by the conversion of prion protein to its abnormal isoform. Studies have shown that ER stress and proteasome impairment occur early in spontaneous prion diseases, contributing to the accumulation of misfolded proteins. The findings suggest a strong correlation between ER stress, proteasome impairment and the neuropathological markers of prion diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Jowita Nowakowska-Golacka, Justyna Czapiewska, Hanna Sominka, Natalia Sowa-Rogozinska, Monika Slominska-Wojewodzka
Summary: EDEM1 is an important regulatory factor involved in APP metabolism, as it recognizes misfolded APP proteins and directs them to cytosol for degradation, leading to decreased secretion of Aβ.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Ji An Kang, Young Joo Jeon
Summary: The endoplasmic reticulum (ER) is crucial for protein synthesis and quality control, requiring significant resources to maintain protein homeostasis. Misfolded proteins pose a threat to proteostasis, which can be regulated through ERAD and ER-phagy processes in the ER.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Mari A. Piirainen, Alexander D. Frey
Summary: Yeasts are widely used for biopharmaceutical production, but N-glycosylated biopharmaceuticals manufactured with yeasts are missing on the market. N-linked glycans play crucial roles in protein properties and folding, but current glycoengineering approaches face challenges.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Anna Signorile, Anna Ferretta, Consiglia Pacelli, Nazzareno Capitanio, Paola Tanzarella, Maria Laura Matrella, Alessio Valletti, Domenico De Rasmo, Tiziana Cocco
Summary: Parkin plays a crucial role in mitochondrial function and calcium balance. Treatment with resveratrol in Parkin-mutant fibroblasts can lead to increased Ca2+ level, modulation of cAMP homeostasis, and restoration of protein expression involved in ER-mitochondria contact points.
Article
Biochemistry & Molecular Biology
Anna Kowalczuk, Krzysztof Marycz, Justyna Kornicka, Sylwia Groborz, Justyna Meissner, Malwina Mularczyk
Summary: In this study, the protective effects of THCV isolated from Cannabis sativa L. against ER stress in adipose-derived mesenchymal stem cells were evaluated. It was found that THCV can prevent subcellular alterations, restore cell functions upon ER stress, and decrease the expression of genes involved in the UPR pathway. These findings suggest that THCV has potential as a therapeutic compound for metabolic diseases associated with ER stress.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biology
Aster Legesse, Nathali Kaushansky, Ilana Braunstein, Haddas Saad, Gerardo Lederkremer, Ami Navon, Ariel Stanhill
Summary: Protein quality control is a crucial process to monitor the folding status of proteins. RNF149, a membrane bound E3 ligase, plays a significant role in maintaining cellular functionality by ubiquitinating non-translocated proteins. Dysfunction of RNF149 leads to disruption of cellular quality control and is associated with pathological conditions related to impaired quality control.
COMMUNICATIONS BIOLOGY
(2023)
Article
Cell Biology
Ron Benyair, Navit Ogen-Shtern, Niv Mazkereth, Ben Shai, Marcelo Ehrlich, Gerardo Z. Lederkremer
MOLECULAR BIOLOGY OF THE CELL
(2015)
Review
Cell Biology
Ron Benyair, Navit Ogen-Shtern, Gerardo Z. Lederkremer
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
(2015)
Article
Gastroenterology & Hepatology
Yoav Lurie, Muriel Webb, Ruth Cytter-Kuint, Shimon Shteingart, Gerardo Z. Lederkremer
WORLD JOURNAL OF GASTROENTEROLOGY
(2015)
Review
Neurosciences
Navit Ogen-Shtern, Tamuz Ben David, Gerardo Z. Lederkremer
Article
Biochemistry & Molecular Biology
Navit Ogen-Shtern, Edward Avezov, Marina Shenkman, Ron Benyair, Gerardo Z. Lederkremer
JOURNAL OF MOLECULAR BIOLOGY
(2016)
Article
Microscopy
Ron Benyair, Gerardo Z. Lederkremer
Article
Cell Biology
Julia Leitman, Marina Shenkman, Yana Gofman, Navit Ogen Shtern, Nir Ben-Tal, Linda M. Hendershot, Gerardo Z. Lederkremer
MOLECULAR BIOLOGY OF THE CELL
(2014)
Review
Biochemistry & Molecular Biology
Marina Shenkman, Gerardo Z. Lederkremer
TRENDS IN BIOCHEMICAL SCIENCES
(2019)
Editorial Material
Biochemistry & Molecular Biology
Haddas Saad, Chaitanya Patel, Gerardo Z. Lederkremer
JOURNAL OF BIOLOGICAL CHEMISTRY
(2019)
Article
Multidisciplinary Sciences
Javier Ganz, Talya Shacham, Maria Kramer, Marina Shenkman, Hagit Eiger, Nitai Weinberg, Ori Iancovici, Somnath Roy, Luba Simhaev, Benny Da'adoosh, Hamutal Engel, Nisim Perets, Yael Barhum, Moshe Portnoy, Daniel Offen, Gerardo Z. Lederkremer
SCIENTIFIC REPORTS
(2020)
Article
Clinical Neurology
Paula Garcia-Huerta, Paulina Troncoso-Escudero, Di Wu, Arun Thiruvalluvan, Marisol Cisternas-Olmedo, Daniel R. Henriquez, Lars Plate, Pedro Chana-Cuevas, Cristian Saquel, Peter Thielen, Kenneth A. Longo, Brad J. Geddes, Gerardo Z. Lederkremer, Neeraj Sharma, Marina Shenkman, Swati Naphade, S. Pablo Sardi, Carlos Spichiger, Hans G. Richter, Felipe A. Court, Kizito Tshitoko Tshilenge, Lisa M. Ellerby, R. Luke Wiseman, Christian Gonzalez-Billault, Steven Bergink, Rene L. Vidal, Claudio Hetz
ACTA NEUROPATHOLOGICA
(2020)
Review
Cell Biology
Chaitanya Patel, Haddas Saad, Marina Shenkman, Gerardo Z. Lederkremer
Review
Biochemistry & Molecular Biology
Talya Shacham, Chaitanya Patel, Gerardo Z. Lederkremer
Summary: With the extension of life span, there is an increasing burden of late-onset neurodegenerative diseases, for which effective treatments are lacking. Strategies to inhibit cellular toxicity and restore homeostasis are drawing increasing attention as gene therapy approaches face difficulties in tackling these diseases. Both inhibition and activation of the PERK pathway have shown benefits in disease models, but the complexity of the pathway and its effects on cell survival or death have led to conflicting results.
Review
Biochemistry & Molecular Biology
Talya Shacham, Neeraj Sharma, Gerardo Z. Lederkremer
FRONTIERS IN MOLECULAR BIOSCIENCES
(2019)
Article
Biology
Marina Shenkman, Efrat Ron, Rivka Yehuda, Ron Benyair, Isam Khalaila, Gerardo Z. Lederkremer
COMMUNICATIONS BIOLOGY
(2018)