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Exclusive expression of the Rab11 effector SH3TC2 in Schwann cells links integrin-α6 and myelin maintenance to Charcot-Marie-Tooth disease type 4C.
The Rab32/BLOC-3-dependent pathway mediates host defense against different pathogens in human macrophages.
Massimiliano Baldassarre, Virtu Solano-Collado, Arda Balci, Rosa A Colamarino, Ivy M Dambuza, Delyth M Reid, Heather M Wilson, Gordon D Brown, Subhankar Mukhopadhyay, Gordon Dougan, Stefania Spanò
Feb 19, 2021
Macrophages provide a first line of defense against microorganisms, and while some mechanisms to kill pathogens such as the oxidative burst are well described, others are still undefined or unknown. Here, we report that the...
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
A high-content endogenous GLUT4 trafficking assay reveals new aspects of adipocyte biology.
Alexis Diaz-Vegas, Dougall M Norris, Sigrid Jall-Rogg, Kristen C Cooke, Olivia J Conway, Amber S Shun-Shion, Xiaowen Duan, Meg Potter, Julian van Gerwen, Harry Jm Baird, Sean J Humphrey, David E James, Daniel J Fazakerley, James G Burchfield
Nov 26, 2022
The RAB11A-Positive Compartment Is a Primary Platform for Autophagosome Assembly Mediated by WIPI2 Recognition of PI3P-RAB11A.
Claudia Puri, Mariella Vicinanza, Avraham Ashkenazi, Matthew J Gratian, Qifeng Zhang, Carla F Bento, Maurizio Renna, Fiona M Menzies, David C Rubinsztein
Jun 01, 2018
Autophagy is a critical pathway that degrades intracytoplasmic contents by engulfing them in double-membraned autophagosomes that are conjugated with LC3 family members. These membranes are specified by phosphatidylinositol...
RAB11A WIPI2 Autophagy autophagy platform macroautophagy mitophagy recycling endosome transferrin receptor Autophagosomes Autophagy Autophagy-Related Proteins Carrier Proteins Endosomes HeLa Cells Humans Membrane Proteins Microtubule-Associated Proteins Phosphate-Binding Proteins Phosphatidylinositol Phosphates Protein Transport Receptors Transferrin rab GTP-Binding Proteins
Griscelli Syndrome Type 2 Sine Albinism
Yuta Ohishi, Sandra Ammann, Vahid Ziaee, Katharina Strege, Miriam Groß, Carla Vazquez Amos, Mohammad Shahrooei, Parisa Ashournia, Anahita Razaghian, Gillian M Griffiths, Stephan Ehl, Mitsunori Fukuda, Nima Parvaneh
Jan 18, 2021
Griscelli syndrome type 2 (GS-2) is an inborn error of immunity characterized by partial albinism and episodes of hemophagocytic lymphohistiocytosis (HLH). It is caused by RAB27A mutations that encode RAB27A, a member of the Rab...
Leukocytes Mononuclear Cell Line COS Cells Animals Humans Albinism Piebaldism rab GTP-Binding Proteins Adaptor Proteins Signal Transducing Membrane Proteins Binding Sites Mutation Missense Adolescent Child Child Preschool Infant Infant Newborn Female Male Lymphohistiocytosis Hemophagocytic rab27 GTP-Binding Proteins Primary Immunodeficiency Diseases Chlorocebus aethiops
ARF6 and Rab11 as intrinsic regulators of axon regeneration.
Adult central nervous system (CNS) axons do not regenerate after injury because of extrinsic inhibitory factors, and a low intrinsic capacity for axon growth. Developing CNS neurons have a better regenerative ability, but lose...
A trimeric Rab7 GEF controls NPC1-dependent lysosomal cholesterol export.
Dick JH van den Boomen, Agata Sienkiewicz, Ilana Berlin, Marlieke LM Jongsma, Daphne M van Elsland, J Paul Luzio, Jacques JC Neefjes, Paul J Lehner
Sep 22, 2020
Biological Transport CRISPR-Cas Systems Cholesterol Cholesterol LDL Endosomes Fluorescent Dyes Genome Human Guanine Nucleotide Exchange Factors HEK293 Cells HeLa Cells Homeostasis Humans Hydroxymethylglutaryl-CoA Synthase Intracellular Signaling Peptides and Proteins Lysosomes Models Biological Multiprotein Complexes Niemann-Pick C1 Protein Protein Binding Protein Multimerization rab GTP-Binding Proteins rab7 GTP-Binding Proteins
Epstein-Barr virus subverts mevalonate and fatty acid pathways to promote infected B-cell proliferation and survival.
Liang Wei Wang, Zhonghao Wang, Ina Ersing, Luis Nobre, Rui Guo, Sizun Jiang, Stephen Trudeau, Bo Zhao, Michael P Weekes, Benjamin E Gewurz
Aug 19, 2019
Epstein-Barr virus (EBV) causes infectious mononucleosis and is associated with multiple human malignancies. EBV drives B-cell proliferation, which contributes to the pathogenesis of multiple lymphomas. Yet, knowledge of how EBV...
Alkyl and Aryl Transferases B-Lymphocytes Cell Proliferation Cell Survival Cholesterol Epstein-Barr Virus Infections Epstein-Barr Virus Nuclear Antigens Fatty Acids Herpesvirus 4 Human Host Microbial Interactions Humans Metabolic Networks and Pathways Mevalonic Acid Proto-Oncogene Proteins c-myc Sterol Regulatory Element Binding Protein 2 Viral Proteins rab GTP-Binding Proteins
Optineurin links myosin VI to the Golgi complex and is involved in Golgi organization and exocytosis.
Daniela A Sahlender, Rhys C Roberts, Susan D Arden, Giulietta Spudich, Marcus J Taylor, J Paul Luzio, John Kendrick-Jones, Folma Buss
Aug 27, 2020
Myosin VI plays a role in the maintenance of Golgi morphology and in exocytosis. In a yeast 2-hybrid screen we identified optineurin as a binding partner for myosin VI at the Golgi complex and confirmed this interaction in a...
Animals Biological Transport CHO Cells Cell Cycle Proteins Chickens Cricetinae Exocytosis Gene Expression Golgi Apparatus HeLa Cells Humans Huntingtin Protein Membrane Glycoproteins Membrane Transport Proteins Myosin Heavy Chains Nerve Tissue Proteins Nuclear Proteins Protein Binding RNA Interference Transcription Factor TFIIIA Transport Vesicles Two-Hybrid System Techniques Viral Envelope Proteins rab GTP-Binding Proteins
Mutations in LRRK2 linked to Parkinson disease sequester Rab8a to damaged lysosomes and regulate transferrin-mediated iron uptake in microglia.
Adamantios Mamais, Jillian H Kluss, Luis Bonet-Ponce, Natalie Landeck, Rebekah G Langston, Nathan Smith, Alexandra Beilina, Alice Kaganovich, Manik C Ghosh, Laura Pellegrini, Ravindran Kumaran, Ioannis Papazoglou, George R Heaton, Rina Bandopadhyay, Nunziata Maio, Changyoun Kim, Matthew J LaVoie, David C Gershlick, Mark R Cookson
Jan 28, 2022
Mutations in leucine-rich repeat kinase 2 (LRRK2) cause autosomal dominant Parkinson disease (PD), while polymorphic LRRK2 variants are associated with sporadic PD. PD-linked mutations increase LRRK2 kinase activity and induce...
Aged Animals Biological Transport Corpus Striatum Gain of Function Mutation HEK293 Cells Humans Iron Leucine-Rich Repeat Serine-Threonine Protein Kinase-2 Lysosomes Male Mice Mice Inbred C57BL Microglia Middle Aged Mutation Parkinson Disease Protein Serine-Threonine Kinases Transferrin Transferrins rab GTP-Binding Proteins
BLOC-1 and BLOC-3 regulate VAMP7 cycling to and from melanosomes via distinct tubular transport carriers.
Megan K Dennis, Cédric Delevoye, Amanda Acosta-Ruiz, Ilse Hurbain, Maryse Romao, Geoffrey G Hesketh, Philip S Goff, Elena V Sviderskaya, Dorothy C Bennett, J Paul Luzio, Thierry Galli, David J Owen, Graça Raposo, Michael S Marks
Aug 18, 2016
Animals Carrier Proteins Cell Membrane Endocytosis Green Fluorescent Proteins Humans Intracellular Signaling Peptides and Proteins Lectins Melanocytes Melanosomes Membrane Glycoproteins Mice Inbred C57BL Mitochondrial Proteins Nerve Tissue Proteins Oxidoreductases Pigmentation Protein Transport Qa-SNARE Proteins R-SNARE Proteins Recombinant Fusion Proteins Transport Vesicles rab GTP-Binding Proteins
The TRAPP complexes
The Transport Protein Particle (TRAPP) complexes are highly conserved multisubunit complexes that act as nucleotide exchange factors (GEFs) for Rab GTPases. They act in both protein secretion and autophagy and have also been...
Late Endosomes Act as mRNA Translation Platforms and Sustain Mitochondria in Axons.
Jean-Michel Cioni, Julie Qiaojin Lin, Anne V Holtermann, Max Koppers, Maximilian AH Jakobs, Afnan Azizi, Benita Turner-Bridger, Toshiaki Shigeoka, Kristian Franze, William A Harris, Christine E Holt
Dec 17, 2018
Local translation regulates the axonal proteome, playing an important role in neuronal wiring and axon maintenance. How axonal mRNAs are localized to specific subcellular sites for translation, however, is not understood. Here...
Charcot-Marie-Tooth type 2B RNA trafficking axon maintenance endosome local protein synthesis lysosome mRNA translation Mitochondria Retinal ganglion cell Animals Axons Endosomes Mitochondria Protein Biosynthesis RNA RNA Messenger Retinal Ganglion Cells Ribosomes Xenopus Proteins Xenopus laevis rab GTP-Binding Proteins rab7 GTP-Binding Proteins
Selective rab11 transport and the intrinsic regenerative ability of CNS axons.
Hiroaki Koseki, Matteo Donegá, Brian YH Lam, Veselina Petrova, Susan van Erp, Giles SH Yeo, Jessica CF Kwok, Charles ffrench-Constant, Richard Eva, James W Fawcett
May 09, 2018
Neurons lose intrinsic axon regenerative ability with maturation, but the mechanism remains unclear. Using an in-vitro laser axotomy model, we show a progressive decline in the ability of cut CNS axons to form a new growth cone...
Inhibition of TBC1D5 activates Rab7a and can enhance the function of the retromer cargo-selective complex.
The retromer complex is a vital component of the endosomal protein sorting machinery necessary for sorting into both the endosome-to-Golgi retrieval pathway and also the endosome-to-cell-surface recycling pathway. Retromer...
Patients with Griscelli syndrome and normal pigmentation identify RAB27A mutations that selectively disrupt MUNC13-4 binding.
Valentina Cetica, Yvonne Hackmann, Samantha Grieve, Elena Sieni, Benedetta Ciambotti, Maria Luisa Coniglio, Daniela Pende, Kimberly Gilmour, Paolo Romagnoli, Gillian M Griffiths, Maurizio Aricò
Jun 05, 2018
BACKGROUND: Familial hemophagocytic lymphohistiocytosis (FHL) is a rare and often fatal disorder characterized by defective cellular cytotoxicity and hyperinflammation, and the only cure known to date is hematopoietic stem cell...
Griscelli syndrome type 2 Hemophagocytic lymphohistiocytosis cytotoxic T lymphocyte familial hemophagocytic lymphohistiocytosis melanophilin natural killer cells Adaptor Proteins Signal Transducing Adolescent Albinism Case-Control Studies Cell Degranulation Cell Line Child Child Preschool Cohort Studies Cytotoxicity Immunologic DNA Mutational Analysis Female Gene Expression Genetic Markers Humans Infant Lymphohistiocytosis Hemophagocytic Male Membrane Proteins Models Molecular Mutation Perforin Phenotype Protein Binding Protein Conformation Skin Pigmentation rab GTP-Binding Proteins rab27 GTP-Binding Proteins
Mirror-symmetric microtubule assembly and cell interactions drive lumen formation in the zebrafish neural rod.
Clare E Buckley, Xiaoyun Ren, Laura C Ward, Gemma C Girdler, Claudio Araya, Mary J Green, Brian S Clark, Brian A Link, Jonathan DW Clarke
Feb 01, 2019
By analysing the cellular and subcellular events that occur in the centre of the developing zebrafish neural rod, we have uncovered a novel mechanism of cell polarisation during lumen formation. Cells from each side of the...
Animals Body Patterning Carrier Proteins Cell Communication Cell Division Cell Movement Cell Polarity Embryo Nonmammalian Green Fluorescent Proteins Luminescent Agents Microtubules Mutation Neural Tube Neurulation Nocodazole Protein Transport Recombinant Fusion Proteins Tubulin Modulators Zebrafish Zebrafish Proteins rab GTP-Binding Proteins
Coordinated assembly and release of adhesions builds apical junctional belts during de novo polarisation of an epithelial tube
Using the zebrafish neural tube as a model, we uncover the in vivo
mechanisms allowing the generation of two opposing apical epithelial
surfaces within the centre of an initially unpolarised, solid organ.
We show that Mpp5a and...