4.5 Article

CSN5 binds to misfolded CFTR and promotes its degradation

期刊

出版社

ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2008.01.010

关键词

CFTR; CSN5; COP9 signalosome; interaction and degradation

向作者/读者索取更多资源

Cystic fibrosis is mainly caused by mutations that interfere with the biosynthetic folding of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The aim of this study was to find cellular proteins interacting with CFTR and regulating its processing. We have used a genetic screen in yeast to identify such proteins and identified CSN5 that interacted with the third cytoplasmic loop of CFTR. CSN5 is the 5th component of the COP9 signalosome, a complex of eight subunits that shares significant homologies to the lid subcomplex of the 26S proteasome and controls the stability of many proteins. The present study shows that CSN5 associates with the core-glycosylated form of CFTR and suggests that this association targets misfolded CFTR to the degradative pathway. Identifying CSN5 as a new component of the degradative pathway is an important step towards the goal of unraveling the sorting between misfolded and correctly folded CFTR proteins. (C) 2008 Elsevier B.V. All rights reserved.

作者

我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。

评论

主要评分

4.5
评分不足

次要评分

新颖性
-
重要性
-
科学严谨性
-
评价这篇论文

推荐

Article Cardiac & Cardiovascular Systems

Natural history and impact of treatment with tafamidis on major cardiovascular outcome-free survival time in a cohort of patients with transthyretin amyloidosis

Melanie Bezard, Mounira Kharoubi, Arnault Galat, Elsa Poullot, Soulef Guendouz, Pascale Fanen, Benoit Funalot, Anissa Moktefi, Jean-Pascal Lefaucheur, Mukedaisi Abulizi, Jean-Francois Deux, Thierry Gendre, Vincent Audard, Khalil el Karoui, Florence Canoui-Poitrine, Amira Zaroui, Emmanuel Itti, Emmanuel Teiger, Violaine Plante-Bordeneuve, Silvia Oghina, Thibaud Damy

Summary: This study found an association between tafamidis treatment and a lower occurrence of cardiovascular outcomes in a real-life population of ATTRwt and ATTRv patients representative of the inclusion criteria of the ATTR-ACT trial.

EUROPEAN JOURNAL OF HEART FAILURE (2021)

Article Allergy

Association analysis of the surfactant protein-C gene to childhood asthma

Malek Nefzi, Imen Wahabi, Sondess Hadj Fredj, Rym Othmani, Rym Dabboubi, Khedija Boussetta, Pascale Fanen, Taieb Messaoud

Summary: This study describes the molecular variability in the SFTPC gene in childhood asthma in the Tunisian population. The T138N polymorphism shows a significant association with asthma, and the 138Asn-186Ser and 138Thr-186Asn haplotypes have a negative association with the disease. This research highlights the genetic variability of the SFTPC gene and its potential role in asthma in Tunisia.

JOURNAL OF ASTHMA (2022)

Article Pharmacology & Pharmacy

Methylprednisolone pulse treatment improves ProSP-C trafficking in twins with SFTPC mutation: An isoform story?

Celine Delestrain, Abdel Aissat, Stephanie Simon, Agathe Tarze, Elodie Duprat, Elodie Nattes, Bruno Costes, Valerie Delattre, Stephanie Finet, Pascale Fanen, Ralph Epaud

Summary: Twins with SP-C gene mutation underwent successful GC treatment, leading to improved proSP-C processing in BALF analysis and a decrease in SFTPC mRNA expression with splicing changes, resulting in the expression of a less stable proSP-C isoform.

BRITISH JOURNAL OF CLINICAL PHARMACOLOGY (2021)

Article Respiratory System

Exon identity influences splicing induced by exonic variants and in silico prediction efficacy

Natacha Martin, Anne Bergougnoux, Nesrine Baatallah, Benoit Chevalier, Jessica Varilh, David Baux, Bruno Costes, Pascale Fanen, Caroline Raynal, Isabelle Sermet-Gaudelus, Emmanuelle Girodon, Magali Taulan-Cadars, Alexandre Hinzpeter

Summary: This study identified 26 out of 65 CFTR variants that enhanced exon skipping, with in silico prediction efficacy ranging from 50% to 66%. Classification of exons based on their basal exon skipping level improved prediction rates up to 80%.

JOURNAL OF CYSTIC FIBROSIS (2021)

Letter Hematology

Multiple thrombosis in a patient with Gardos channelopathy and a new KCNN4 mutation

Lamisse Mansour-Hendili, Stephane Egee, David Monedero-Alonso, Guillaume Bouyer, Bertrand Godeau, Bouchra Badaoui, Ariane Lunati, Clara Noizat, Abdelrazak Aissat, Laurent Kiger, Chadia Mekki, Veronique Picard, Stephane Moutereau, Pascale Fanen, Pablo Bartolucci, Loic Garcon, Frederic Galacteros, Benoit Funalot

AMERICAN JOURNAL OF HEMATOLOGY (2021)

Review Biochemistry & Molecular Biology

Small Hsps as Therapeutic Targets of Cystic Fibrosis Transmembrane Conductance Regulator Protein

Stephanie Simon, Abdel Aissat, Fanny Degrugillier, Benjamin Simonneau, Pascale Fanen, Andre-Patrick Arrigo

Summary: Human small heat shock proteins, including HspB1, HspB4, and HspB5, play crucial roles in regulating cellular processes related to Cystic Fibrosis (CF). While HspB1 and HspB4 promote the degradation of CFTR mutants, HspB5 enhances the transport and function of CFTR mutant at the plasma membrane. Additionally, HspB5 molecules can increase the cellular efficiency of current CF therapeutic molecules.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

Article Genetics & Heredity

Prenatal Ultrasound Suspicion of Cystic Fibrosis in a Multiethnic Population: Is Extensive CFTR Genotyping Needed?

Chadia Mekki, Abdel Aissat, Veronique Mirlesse, Sophie Mayer Lacrosniere, Elsa Eche, Annick Le Floch, Sandra Whalen, Cecile Prud'Homme, Christelle Remus, Benoit Funalot, Vanina Castaigne, Pascale Fanen, Alix de Becdelievre

Summary: This study shows that fetal ultrasound bowel abnormalities can unexpectedly reveal cystic fibrosis (CF) in families without a CF history. A comprehensive study of the CFTR gene is recommended for cases with bowel abnormalities to identify potential mutations, even in populations without well-known frequent pathogenic variants.
Article Biochemistry & Molecular Biology

Structure-Based Understanding of ABCA3 Variants

Marion Onnee, Pascale Fanen, Isabelle Callebaut, Alix de Becdelievre

Summary: ABCA3 protein plays a crucial role in pulmonary surfactant biosynthesis and is associated with recessive pulmonary disorders. Using 3D structure information, researchers modeled the protein and pinpointed known pathogenic missense variants, providing insights into the possible impact of these variants in the protein's structure and function.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

Article Cardiac & Cardiovascular Systems

Assessing Cardiac Amyloidosis Subtypes by Unsupervised Phenotype Clustering Analysis

Louis Bonnefous, Mounira Kharoubi, Melanie Bezard, Silvia Oghina, Fabien Le Bras, Elsa Poullot, Valerie Molinier-Frenkel, Pascale Fanen, Jean-Francois Deux, Vincent Audard, Emmanuel Itti, Thibaud Damy, Etienne Audureau

Summary: Clustering analysis identified 7 distinct clinical profiles among suspected CA patients, indicating areas for improvement in amyloidosis diagnosis and prognosis stratification based on associated risk factors.

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2021)

Article Medicine, General & Internal

Echocardiographic Patterns of Left Ventricular Diastolic Function in Cardiac Amyloidosis: An Updated Evaluation

Silvia Oghina, Wulfran Bougouin, Mounira Kharoubi, Louis Bonnefous, Arnault Galat, Soulef Guendouz, Melanie Bezard, Fabien Le Bras, Jean-Francois Deux, Emmanuel Itti, Anissa Moktefi, Pascale Fanen, Emmanuel Teiger, Dania Mohty, Thibaud Damy, Diane Bodez

Summary: All LVDF patterns can be observed in CA, and one quarter of CA patients have a better prognosis with grade I LVDF.

JOURNAL OF CLINICAL MEDICINE (2021)

Article Medicine, General & Internal

Prognostic Value of N-Terminal Pro-Brain Natriuretic Peptide and High-Sensitivity Troponin T Levels in the Natural History of Transthyretin Amyloid Cardiomyopathy and Their Evolution after Tafamidis Treatment

Silvia Oghina, Constant Josse, Melanie Bezard, Mounira Kharoubi, Marc-Antoine Delbarre, Damien Eyharts, Amira Zaroui, Soulef Guendouz, Arnault Galat, Luc Hittinger, Pascale Fanen, Emmanuel Teiger, Nadir Mouri, Francois Montestruc, Thibaud Damy

Summary: The study assessed the evolution and prognostic value of NT-proBNP and cTnT-HS in ATTR-CA patients before and after tafamidis treatment. The results showed that patients with increasing NT-proBNP had an increased risk of adverse events, but tafamidis treatment could stabilize these biomarker levels.

JOURNAL OF CLINICAL MEDICINE (2021)

Article Cardiac & Cardiovascular Systems

Prevalence and determinants of iron deficiency in cardiac amyloidosis

Antoine Jobbe-Duval, Melanie Bezard, Stephane Moutereau, Mounira Kharoubi, Silvia Oghina, Amira Zaroui, Arnault Galat, Coraline Chalard, Elisabeth Hugon-Vallet, Francois Lemonnier, Damien Eyharts, Elsa Poulot, Pascale Fanen, Benoit Funalot, Valerie Molinier-Frenkel, Vincent Audard, Luc Hittinger, Marc Antoine Delbarre, Emmanuel Teiger, Thibaud Damy

Summary: Iron deficiency is common in patients with cardiac amyloidosis, regardless of subtype. Patients are more likely to have iron deficiency if diagnosed with ATTR, diabetic, and/or treated with aspirin. The benefit of intravenous iron therapy for iron deficiency in cardiac amyloidosis needs further study on morbidity and mortality.

ESC HEART FAILURE (2022)

Article Cardiac & Cardiovascular Systems

History of extracardia/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis

Mounira Kharoubi, Melanie Bezard, Arnault Galat, Fabien Le Bras, Elsa Poullot, Valerie Molinier-Frenkel, Pascale Fanen, Benoit Funalot, Anissa Moktefi, Jean-Pascal Lefaucheur, Mukedaisi Abulizi, Jean-Francois Deux, Francois Lemonnier, Soulef Guendouz, Coraline Chalard, Amira Zaroui, Vincent Audard, Emilie Bequignon, Diane Bodez, Emmanuel Itti, Luc Hittinger, Etienne Audureau, Emmanuel Teiger, Silvia Oghina, Thibaud Damy

Summary: This study reveals the importance of amyloidosis type and progression in diagnostic delay and prognosis, highlighting the need for physicians to remain vigilant towards extracardiac and cardiac events for significantly improving early diagnosis of amyloidosis.

ESC HEART FAILURE (2021)

Letter Biochemistry & Molecular Biology

Instability of Mature ABCA3 Protein: Toward a New Classification of ABCA3 Mutations?

Marion Onnee, Benedicte Duriez, Stephanie Simon, Claire Szczepaniak, Aurelie Guguin, Ralph Epaud, Samia Hamouda, Pascale Fanen, Alix de Becdelievere

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2022)

Article Cardiac & Cardiovascular Systems

History of extracardiac cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis

Mounira Kharoubi, Melanie Bezard, Arnault Galat, Fabien Le Bras, Elsa Poullot, Valerie Molinier-Frenkel, Pascale Fanen, Benoit Funalot, Anissa Moktefi, Jean-Pascal Lefaucheur, Mukedaisi Abulizi, Jean-Francois Deux, Francois Lemonnier, Soulef Guendouz, Coraline Chalard, Amira Zaroui, Vincent Audard, Emilie Bequignon, Diane Bodez, Emmanuel Itti, Luc Hittinger, Etienne Audureau, Emmanuel Teiger, Silvia Oghina, Thibaud Damy

Summary: This study compared AECE and ACE between different types of CA, finding that the age of onset for the first AECE and ACE was lower for AL and ATTRv patients compared to ATTRwt patients. In AL and ATTRwt, a shorter delay between the onset of the first AECE or ACE and diagnosis was associated with reduced survival rate.

ESC HEART FAILURE (2021)

Article Biochemistry & Molecular Biology

Hsc70 phosphorylation patterns and calmodulin regulate AP2 Clathrin-Coated-Vesicle life span for cell adhesion protein transport

G. F. Senguel, R. Mishra, E. Candiello, P. Schu

Summary: AP2 forms AP2 CCV with clathrin and other coat proteins, and synapses contain different types of CCV. The stability and composition of CCV are regulated by various factors, including Hsc70 and phosphorylation patterns. The knockout of the AP1/O1B complex disrupts synaptic vesicle recycling and endosomal protein sorting, leading to upregulation of endocytosis. Stable CCV, termed stCCV, have distinct characteristics and specialized functions in synaptic plasticity. The phosphorylation of Hsc70 and the levels of kinases play a crucial role in regulating the stability and disassembly of clathrin in CCV.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Enhanced capacity for CaMKII signaling mitigates calcium release related contractile fatigue with high intensity exercise

Martin Fluck, Colline Sanchez, Vincent Jacquemond, Christine Berthier, Marie-Noelle Giraud, Daniel Jacko, Kathe Bersiner, Sebastian Gehlert, Guus Baan, Richard T. Jaspers

Summary: Enhancing CaMKII signaling improves fatigue resistance and contractile characteristics of skeletal muscle by enhancing calcium release.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Letter Biochemistry & Molecular Biology

SQSTM1/p62 inhibition impairs pro-survival signaling in hypoxic human dendritic cells

Federica Coppola, Sara Monaci, Alessandro Falsini, Carlo Aldinucci, Irene Filippi, Daniela Rossi, Fabio Carraro, Antonella Naldini

Summary: The adaptor protein p62 plays a crucial role in maintaining the survival of dendritic cells (DCs) under hypoxic conditions by preserving Erk1/2 phosphorylation and reducing AMPK activation, thus extending their lifespan to ensure their functions in hypoxic microenvironments.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

High-throughput analysis of glycan sorting into extracellular vesicles

Jenifer Pendiuk Goncalves, Jorvani Cruz Villarreal, Sierra A. Walker, Xuan Ning Sharon Tan, Chad Borges, Joy Wolfram

Summary: This study used a mass spectrometry-based approach to assess the differences in glycan features between extracellular vesicles (EVs) and originating cells. The results showed that EVs selectively enriched specific glycan features, particularly those associated with binding to the extracellular matrix. The study also found differences in EV glycan sorting between different metastatic cell lines and mouse models, indicating a potential role of glycan diversity in the metastatic process.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

SPOP promotes CREB5 ubiquitination to inhibit MET signaling in liver cancer

De-ao Gong, Peng Zhou, Wen-yi Chang, Jia-yao Yang, Yan-lai Zhang, Ai-long Huang, Ni Tang, Kai Wang

Summary: Liver cancer, ranked sixth globally, is a major contributor to cancer-related mortality. Metastasis is the main cause of treatment failure and deaths in liver cancer. The SPOP-CREB5-MET axis plays a significant role in liver cancer metastasis.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Glioma-derived S100A9 polarizes M2 microglia to inhibit CD8+T lymphocytes for immunosuppression via αv133 integrin/AKT1/TGF131

Ning Huang, Jun Tang, Xiaoyao Yi, Maoxin Zhang, Bin Li, Yuan Cheng, Jin Chen

Summary: This study reveals that glioma-derived S100A9 can induce microglial M2 polarization, inhibit CD8+ T lymphocytes, and promote immunosuppression. The mechanism is related to the interaction with alpha v133 integrin and subsequent activation of AKT1 in microglia. The expression of S100A9 is positively associated with CD206 expression and negatively correlated with CD8+ T lymphocyte accumulation in the TME, suggesting a potential role of S100A9 in regulating the tumor microenvironment and immune evasion in glioma.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Inhibition of autophagy initiation: A novel strategy for oral squamous cell carcinomas

Yomna S. Abd El-Aziz, Matthew J. McKay, Mark P. Molloy, Betty McDowell, Elizabeth Moon, Loretta Sioson, Amy Sheen, Angela Chou, Anthony J. Gill, Patric J. Jansson, Sumit Sahni

Summary: This study identified a novel combination of autophagy inhibitors that can effectively inhibit the proliferation of oral squamous cell carcinoma (OSCC) cells, including both chemosensitive and chemoresistant cells. This research is important for the development of new therapies for advanced OSCC tumors.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Paxillin/HDAC6 regulates microtubule acetylation to promote directional migration of keratinocytes driven by electric fields

Luojia Liu, Xiaoqiang Liu, Ying Chen, Meng Kong, Jinghong Zhang, Min Jiang, Hongling Zhou, Jinrui Yang, Xu Chen, Ze Zhang, Chao Wu, Xupin Jiang, Jiaping Zhang

Summary: Our study revealed that the Paxillin/HDAC6 signaling pathway regulates microtubule acetylation in electric field-guided keratinocyte migration.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

The bacterial magnesium transporter MgtA reveals highly selective interaction with specific cardiolipin species

Julia Weikum, Jeroen F. van Dyck, Saranya Subramani, David P. Klebl, Merete Storflor, Stephen P. Muench, Soren Abel, Frank Sobott, J. Preben Morth

Summary: The study reveals the complex interaction between bacterial magnesium transporter A (MgtA) and cardiolipin 18:1 and cardiolipin 16:0, highlighting the importance of lipid environment in protein activity and stability. Further understanding of Mg2+ homeostasis in bacteria will provide insights into bacterial infections.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Lanosterol elevates cytoprotective response through induced-proteasomal degradation of aberrant proteins

Sumit Kinger, Yuvraj Anandrao Jagtap, Ankur Rakesh Dubey, Prashant Kumar, Akash Choudhary, Rohan Dhiman, Vijay Kumar Prajapati, Deepak Chitkara, Krishna Mohan Poluri, Amit Mishra

Summary: Efficient protein synthesis and quality control mechanisms are crucial for maintaining proteostasis and preventing neurodegeneration. This study demonstrates that treating cells with Lanosterol can enhance the proteolytic activity of Proteasome and promote the removal of misfolded proteins, suggesting a potential therapeutic approach for abnormal protein accumulation.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Disorders in the CMG helicase complex increase the proliferative capacity and delay chronological aging of budding yeast

Karolina Stepien, Adrianna Skoneczna, Monika Kula-Maximenko, Lukasz Jurczyk, Mateusz Molon

Summary: The replication of DNA requires a complex machinery called the replisome, which is highly conserved across species. One crucial component of the replisome is the CMG helicase complex, which unwinds DNA and coordinates the assembly and function of other replisome components. In this study, the impact of the absence of one copy of the CMG complex genes on the physiology and aging of yeast cells was investigated. The findings showed disruptions in the cell cycle, extended doubling times, and alterations in the biochemical profile of these cells. Importantly, it was found that heterozygous cells for CMG helicase genes exhibited increased reproductive potential and delayed aging. The study also highlighted potential therapeutic targets for cancer treatment using yeast.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Replacement of Lys27 by asparagine in the SERCA regulator myoregulin: A Ca2+affinity modulator or a catalytic activity switch?

Nishadh Rathod, Guadalupe Guerrero-Serna, Howard S. Young, L. Michel Espinoza-Fonseca

Summary: This study reveals that replacing Lys27 with Asn enhances the inhibitory potency of MLN without affecting SERCA's affinity for Ca2+. The findings suggest that the SERCA site modulating Ca2+ affinity also functions as a catalytic activity switch.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Interplay between SUMO1-related SUMOylation and phosphorylation of p65 promotes hepatocellular carcinoma progression

Can Jiang, Chunyang Zhang, Min Dai, Fuyan Wang, Sa Xu, Dan Han, Yanyan Wang, Yajie Cao, Yanyan Liang, Ziyu Zhang, Lina Yan, Yujun Shen, Kewu He, Yuxian Shen, Jun Liu

Summary: The phosphorylation of p65 and the expression of SUMO1 are increased in cancer tissues of HCC patients, and there is a positive correlation between SUMO1 and phosphorylated p65. SUMOylation of p65 by SUMO1 promotes p65 nuclear import and enhances NF-xB activity. Both SUMOylation and phosphorylation of p65 increase the viability and invasion of hepatoma cells, and decrease cell apoptosis.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Protein tyrosine phosphatase 1B is a regulator of alpha-actinin4 in the glomerular podocyte

Ming-Fo Hsu, Yoshihiro Ito, Jai Prakash Singh, Shu-Fang Hsu, Alan Wells, Kuang-Yu Jen, Tzu-Ching Meng, Fawaz G. Haj

Summary: This study identified alpha-actinin4 as a novel substrate of PTP1B in podocytes and demonstrated their interaction in regulating podocyte function. Targeting PTP1B and alpha-actinin4 could be a potential therapeutic approach for podocyte injury.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)

Article Biochemistry & Molecular Biology

Opposite regulation of glycogen metabolism by cAMP produced in the cytosol and at the plasma membrane

Paulo F. V. Bizerra, Eduardo H. Gilglioni, Hang Lam Li, Simei Go, Ronald P. J. Oude Elferink, Arthur J. Verhoeven, Jung -Chin Chang

Summary: This study investigates the role of cyclic AMP (cAMP) in glycogen metabolism and reveals that cAMP regulates glycogenolysis in opposite directions depending on its site of synthesis within cells and downstream effectors. The canonical tmAC-cAMP-PKA signaling promotes glycogenolysis, while the non-canonical sAC-cAMP-Epac1 signaling suppresses glycogenolysis. This highlights the importance of cAMP microdomain organization for distinct metabolic regulation.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2024)