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Artificial miRNAs Reduce Human Mutant Huntingtin Throughout the Striatum in a Transgenic Sheep Model of Huntington's Disease.
Edith L Pfister, Natalie DiNardo, Erica Mondo, Florie Borel, Faith Conroy, Cara Fraser, Gwladys Gernoux, Xin Han, Danjing Hu, Emily Johnson, Lori Kennington, PengPeng Liu, Suzanne J Reid, Ellen Sapp, Petr Vodicka, Tim Kuchel, A Jennifer Morton, David Howland, Richard Moser, Miguel Sena-Esteves, Guangping Gao, Christian Mueller, Marian DiFiglia, Neil Aronin
Sep 20, 2018
Huntington's disease (HD) is a fatal neurodegenerative disease caused by a genetic expansion of the CAG repeat region in the huntingtin (HTT) gene. Studies in HD mouse models have shown that artificial miRNAs can reduce mutant...
AAV Huntington's disease RNAi large animal models Animals Animals Genetically Modified Dependovirus Disease Models Animal Electrolytes Genetic Vectors Genome Viral Humans Huntingtin Protein Huntington Disease Immunoassay Injections Kidney Liver MicroRNAs Microglia Mutant Proteins Neostriatum Neurons RNA Messenger Sheep
Functional Studies of Missense TREM2 Mutations in Human Stem Cell-Derived Microglia.
Philip W Brownjohn, James Smith, Ravi Solanki, Ebba Lohmann, Henry Houlden, John Hardy, Sabine Dietmann, Frederick J Livesey
Jun 04, 2018
The derivation of microglia from human stem cells provides systems for understanding microglial biology and enables functional studies of disease-causing mutations. We describe a robust method for the derivation of human...
Recombinant amyloid beta-peptide production by coexpression with an affibody ligand.
Bertil Macao, Wolfgang Hoyer, Anders Sandberg, Ann-Christin Brorsson, Christopher M Dobson, Torleif Härd
Jun 14, 2011
Structural insights into the mechanism of negative regulation of single-box high mobility group proteins by the acidic tail domain.
Katherine Stott, Matthew Watson, Mark J Bostock, Simon A Mortensen, Andrew Travers, Klaus D Grasser, Jean O Thomas
Apr 13, 2015
Acidic Regulatory Domain Casein Kinase 2 (CK2) Chromatin HMGB Protein Intrinsically Disordered Protein Nuclear Magnetic Resonance (NMR) Paramagnetic Relaxation Enhancement Phosphorylation Protein Kinase C Protein Kinase C (PKC) Animals Drosophila Proteins Drosophila melanogaster High Mobility Group Proteins Magnetic Resonance Spectroscopy Models Molecular Mutant Proteins Phosphorylation Plant Proteins Protein Structure Tertiary Structure-Activity Relationship Zea mays
A Single Cysteine Residue in the Translocation Pathway of the Mitosomal ADP/ATP Carrier from Cryptosporidium parvum Confers a Broad Nucleotide Specificity.
Martin S King, Sotiria Tavoulari, Vasiliki Mavridou, Alannah C King, John Mifsud, Edmund RS Kunji
Nov 30, 2020
Cryptosporidiumparvum is a clinically important eukaryotic parasite that causes the disease cryptosporidiosis, which manifests with gastroenteritis-like symptoms. The protist has mitosomes, which are organelles of mitochondrial...
SLC25 mitochondrial carrier family Adenine nucleotide translocase Adenine nucleotide translocator substrate binding site substrate specificity and selectivity Amino Acid Sequence Atractyloside Bongkrekic Acid Cryptosporidium parvum Cysteine Lactococcus lactis Mitochondria Mitochondrial ADP ATP Translocases Models Molecular Mutant Proteins Nucleotides Phylogeny Protein Translocation Systems Saccharomyces cerevisiae Saccharomyces cerevisiae Proteins Structure-Activity Relationship Substrate Specificity
Short-term diabetic hyperglycemia suppresses celiac ganglia neurotransmission, thereby impairing sympathetically mediated glucagon responses.
Short-term hyperglycemia suppresses superior cervical ganglia neurotransmission. If this ganglionic dysfunction also occurs in the islet sympathetic pathway, sympathetically mediated glucagon responses could be impaired. Our...
WLDS celiac ganglia fos Glucagon nicotinic acetylcholine receptors Norepinephrine sympathetic nervous system Animals Diabetes mellitus Type 1 Down-Regulation Electric Stimulation Ganglia Sympathetic Ganglionic Stimulants Glucagon Hyperglycemia Islets of Langerhans Male Mutant Proteins Nerve Tissue Proteins Neurons Nicotinic Agonists Norepinephrine Proto-Oncogene Proteins c-fos Rats Sprague-Dawley Rats Transgenic Rats Wistar Receptors Nicotinic Synaptic Transmission Wallerian degeneration
Interferon-β-induced miR-1 alleviates toxic protein accumulation by controlling autophagy.
Camilla Nehammer, Patrick Ejlerskov, Sandeep Gopal, Ava Handley, Leelee Ng, Pedro Moreira, Huikyong Lee, Shohreh Issazadeh-Navikas, David C Rubinsztein, Roger Pocock
Dec 10, 2019
Appropriate regulation of autophagy is crucial for clearing toxic proteins from cells. Defective autophagy results in accumulation of toxic protein aggregates that detrimentally affect cellular function and organismal survival....
C. elegans Autophagy Biochemistry chemical biology Genetics Genomics human cells mammalian cells microRNA protein aggregation 3' Untranslated Regions Animals Autophagy Base Sequence Caenorhabditis elegans Caenorhabditis elegans Proteins GTPase-Activating Proteins HeLa Cells Humans Huntingtin Protein Interferon-beta Mice MicroRNAs Mutant Proteins Peptides Protein Aggregates RNA Messenger rab GTP-Binding Proteins rab7 GTP-Binding Proteins
SARS-CoV-2 evolution during treatment of chronic infection
Steven A Kemp, Dami A Collier, Rawlings P Datir, Isabella ATM Ferreira, Salma Gayed, Aminu Jahun, Myra Hosmillo, Chloe Rees-Spear, Petra Mlcochova, Ines Ushiro Lumb, David J Roberts, Anita Chandra, Nigel Temperton, The CITIID-NIHR BioResource COVID-19 Collaboration, The COVID-19 Genomics UK (COG-UK) Consortium, Katherine Sharrocks, Elizabeth Blane, Yorgo Modis, Kendra E Leigh, John AG Briggs, Marit J van Gils, Kenneth GC Smith, John R Bradley, Chris Smith, Rainer Doffinger, Lourdes Ceron-Gutierrez, Gabriela Barcenas-Morales, David D Pollock, Richard A Goldstein, Anna Smielewska, Jordan P Skittrall, Theodore Gouliouris, Ian G Goodfellow, Effrossyni Gkrania-Klotsas, Christopher JR Illingworth, Laura E McCoy, Ravindra K Gupta
Jan 28, 2021
Adenosine Monophosphate Aged Alanine Antibodies Neutralizing Antibodies Viral covid-19 Chronic Disease evolution Molecular Genome Viral High-Throughput Nucleotide Sequencing Humans Immune Evasion Immune Tolerance Immunization Passive Immunosuppression Therapy Male Mutagenesis Mutant Proteins Mutation Phylogeny SARS-CoV-2 Spike Glycoprotein Coronavirus Time Factors Viral Load Virus Shedding COVID-19 Drug Treatment COVID-19 Serotherapy
The activating mutation R201C in GNAS promotes intestinal tumourigenesis in Apc(Min/+) mice through activation of Wnt and ERK1/2 MAPK pathways.
Somatically acquired, activating mutations of GNAS, the gene encoding the stimulatory G-protein Gsalpha subunit, have been identified in kidney, thyroid, pituitary, leydig cell, adrenocortical and, more recently, in colorectal...
Adenoma Adenomatous Polyposis Coli Protein Alleles Animals Cell Differentiation Cell Proliferation Chromogranins Colorectal Neoplasms Cyclic AMP Disease Progression Epithelial Cells Female GTP-Binding Protein alpha Subunits Gs Gene Expression Regulation Neoplastic Genetic Loci Humans Intestinal Mucosa MAP Kinase Signaling System Male Membrane Glycoproteins Mice Mitogen-Activated Protein Kinase 1 Mitogen-Activated Protein Kinase 3 Mutant Proteins Mutation Organ Specificity Phosphoric Diester Hydrolases Promoter Regions Genetic Stem Cells Substrate Specificity Up-Regulation Wnt Proteins
Targeting codon 158 p53-mutant cancers via the induction of p53 acetylation.
Li Ren Kong, Richard Weijie Ong, Tuan Zea Tan, Nur Afiqah Binte Mohamed Salleh, Matan Thangavelu, Jane Vin Chan, Lie Yong Judice Koh, Giridharan Periyasamy, Jieying Amelia Lau, Thi Bich Uyen Le, Lingzhi Wang, MiYoung Lee, Srinivasaraghavan Kannan, Chandra S Verma, Chwee Ming Lim, Wee Joo Chng, David P Lane, Ashok Venkitaraman, Huynh The Hung, Chit Fang Cheok, Boon Cher Goh
May 15, 2020
Gain of function (GOF) DNA binding domain (DBD) mutations of TP53 upregulate chromatin regulatory genes that promote genome-wide histone methylation and acetylation. Here, we therapeutically exploit the oncogenic GOF mechanisms...
Acetylation Animals Antineoplastic Agents Apoptosis Caspase 3 Cell Line Tumor Cell Proliferation cisplatin Codon Epigenesis Genetic Gain of Function Mutation Gene Expression Regulation Neoplastic Humans Hydroxamic Acids Mice SCID Models Biological Mutant Proteins Mutation NF-kappa B Neoplasms Nucleotide Motifs Poly(ADP-ribose) Polymerases Protein Binding Protein Isoforms Sulfonamides Topotecan Tumor Suppressor Protein p53 Ubiquitin-Protein Ligases Xenograft Model Antitumor Assays
Native-state stability determines the extent of degradation relative to secretion of protein variants from Pichia pastoris.
Graham Whyteside, Marcos JC Alcocer, Janet R Kumita, Christopher M Dobson, Maria Lazarou, Richard J Pleass, David B Archer
Aug 19, 2020
We have investigated the relationship between the stability and secreted yield of a series of mutational variants of human lysozyme (HuL) in Pichia pastoris. We show that genes directly involved in the unfolded protein response...
Epistatic interactions can moderate the antigenic effect of substitutions in haemagglutinin of influenza H3N2 virus.
Björn F Koel, David F Burke, Stefan van der Vliet, Theo M Bestebroer, Guus F Rimmelzwaan, Albert DME Osterhaus, Derek J Smith, Ron AM Fouchier
May 13, 2019
We previously showed that single amino acid substitutions at seven positions in haemagglutinin determined major antigenic change of influenza H3N2 virus. Here, the impact of two such substitutions was tested in 11 representative...
Drosophila phosphatidylinositol-4 kinase fwd promotes mitochondrial fission and can suppress Pink1/parkin phenotypes.
Animals Cytoskeletal Proteins Disease Models Animal Drosophila Proteins Drosophila melanogaster GTP-Binding Proteins Gene Expression Regulation Humans Locomotion Mitochondria Mitochondrial Dynamics mitophagy Mutant Proteins Nerve Degeneration Parkinson Disease Phosphotransferases (Alcohol Group Acceptor) Protein Serine-Threonine Kinases Ubiquitin-Protein Ligases
The function of phosphatidylinositol 5-phosphate 4-kinase γ (PI5P4Kγ) explored using a specific inhibitor that targets the PI5P-binding site.
Jonathan H Clarke, Maria-Luisa Giudici, John E Burke, Roger L Williams, David J Maloney, Juan Marugan, Robin F Irvine
Feb 18, 2015
Amino Acid Substitution Animals Binding Sites Cell Differentiation Cell Line Cell Membrane Cell Polarity Deuterium Exchange Measurement Enzyme Inhibitors Isoenzymes Kidney Cortex Mice Models Molecular Mutant Proteins Phosphatidylinositol Phosphates Phosphotransferases (Alcohol Group Acceptor) Protein Conformation Protein Transport Quinazolines RNA Interference Recombinant Proteins Thiophenes
The JAK-STAT signaling pathway is differentially activated in CALR-positive compared with JAK2V617F-positive ET patients.
This is a metadata record relating to an article that cannot be shared due to publisher copyright.
Amino Acid Substitutions That Affect Receptor Binding and Stability of the Hemagglutinin of Influenza A/H7N9 Virus.
Eefje JA Schrauwen, Mathilde Richard, David F Burke, Guus F Rimmelzwaan, Sander Herfst, Ron AM Fouchier
Mar 14, 2016
Polyglutamine tracts regulate autophagy
DC Rubinsztein, A Ashkenazi, CF Bento, T Ricketts, M Vicinanza, F Siddiqi, M Pavel, F Squitieri, MC Haardenberg, S Imarisio, FM Menzies
Aug 08, 2017
Mutant IP3 receptors attenuate store-operated Ca2+ entry by destabilizing STIM-Orai interactions in Drosophila neurons.
Sumita Chakraborty, Bipan K Deb, Tetyana Chorna, Vera Konieczny, Colin W Taylor, Gaiti Hasan
Oct 25, 2016
Ca2+ signalling Drosophila IP3 receptor Orai STIM Store-operated Ca2+ entry Animals Calcium Calcium Signaling Cell Membrane Chickens Drosophila Proteins Drosophila melanogaster Inositol 1 4 5-Trisphosphate Receptors Models Biological Mutant Proteins Neurons ORAI1 Protein Stromal Interaction Molecule 1 Vertebrates
The V86M mutation in HIV-1 capsid confers resistance to TRIM5α by abrogation of cyclophilin A-dependent restriction and enhancement of viral nuclear import.
Maxime Veillette, Katsiaryna Bichel, Paulina Pawlica, Stefan MV Freund, Mélodie B Plourde, Quang Toan Pham, Carlos Reyes-Moreno, Leo C James, Lionel Berthoux
Mar 06, 2013
Interplay between partner and ligand facilitates the folding and binding of an intrinsically disordered protein.
Joseph M Rogers, Vladimiras Oleinikovas, Sarah L Shammas, Chi T Wong, David De Sancho, Christopher M Baker, Jane Clarke
Oct 20, 2014
BCL-2 Protein Folding coarse-grained simulation Protein–protein interactions stopped flow Intrinsically Disordered Proteins Kinetics Ligands Molecular Dynamics Simulation Mutant Proteins Mutation Myeloid Cell Leukemia Sequence 1 Protein Protein Binding Protein Folding Protein Structure Secondary Proto-Oncogene Proteins
Transcript availability dictates the balance between strand-asynchronous and strand-coupled mitochondrial DNA replication.
Tricia J Cluett, Gokhan Akman, Aurelio Reyes, Lawrence Kazak, Alice Mitchell, Stuart R Wood, Antonella Spinazzola, Johannes N Spelbrink, Ian J Holt
Nov 21, 2018
Mammalian mitochondria operate multiple mechanisms of DNA replication. In many cells and tissues a strand-asynchronous mechanism predominates over coupled leading and lagging-strand DNA synthesis. However, little is known of the...
Wild-type sTREM2 blocks Aβ aggregation and neurotoxicity, but the Alzheimer's R47H mutant increases Aβ aggregation.
Anna Vilalta, Ye Zhou, Jean Sevalle, Jennifer K Griffin, Kanayo Satoh, David H Allendorf, Suman De, Mar Puigdellívol, Arturas Bruzas, Miguel A Burguillos, Roger B Dodd, Fusheng Chen, Yalun Zhang, Patrick Flagmeier, Lisa-Maria Needham, Masahiro Enomoto, Seema Qamar, James Henderson, Jochen Walter, Paul E Fraser, David Klenerman, Steven F Lee, Peter St George-Hyslop, Guy C Brown
Apr 07, 2021
Drosophila MARF1 ensures proper oocyte maturation by regulating nanos expression.
Meiosis and oocyte maturation are tightly regulated processes. The meiosis arrest female 1 (MARF1) gene is essential for meiotic progression in animals; however, its detailed function remains unclear. In this study, we examined...
Amino Acid Motifs Animals Cell Cycle Proteins Cell Differentiation Cell Fusion Conserved Sequence Cyclin B Down-Regulation Drosophila Proteins Drosophila melanogaster Female Gene Expression Regulation Meiosis Mutant Proteins Mutation Oocytes Oogenesis Ovary Protein Binding RNA Messenger RNA-Binding Proteins Thioredoxins
Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB.
Benoit D Roussel, Timothy M Newton, Elke Malzer, Nikol Simecek, Imran Haq, Sally E Thomas, Marian L Burr, Paul J Lehner, Damian C Crowther, Stefan J Marciniak, David A Lomas
May 18, 2018
Mutants of neuroserpin are retained as polymers within the endoplasmic reticulum (ER) of neurones to cause the autosomal dominant dementia familial encephalopathy with neuroserpin inclusion bodies or FENIB. The cellular...
Animals Cells Cultured Cholesterol Disease Models Animal Drosophila melanogaster Endoplasmic Reticulum Epilepsies Myoclonic HeLa Cells Heredodegenerative Disorders Nervous System Humans Mice Mutant Proteins Neurons Neuropeptides Polymers Protein Unfolding Serpins Signal Transduction Ubiquitin-Conjugating Enzymes Ubiquitin-Protein Ligases Unfolded Protein Response
Dual binding motifs underpin the hierarchical association of perilipins1-3 with lipid droplets.
Dalila Ajjaji, Kalthoum Ben M'barek, Michael L Mimmack, Cheryl England, Haya Herscovitz, Liang Dong, Richard G Kay, Satish Patel, Vladimir Saudek, Donald M Small, David B Savage, Abdou Rachid Thiam
Feb 04, 2019
Lipid droplets (LDs) in all eukaryotic cells are coated with at least one of the perilipin (Plin) family of proteins. They all regulate key intracellular lipases but do so to significantly different extents. Where more than one...
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