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AutoantibodiesAquaporin 1Aquaporin 5Aquaporin 3Aquaporin 4AquaporinsAquaporin 2Aquaporin 6AutoantigensAntibodies, AntinuclearWaterAutoimmune DiseasesLupus Erythematosus, SystemicImmunoglobulin GMercuric ChlorideAutoimmunityAntibody SpecificityPemphigusOsmosisBlood Group AntigensAquaglyceroporinsEnzyme-Linked Immunosorbent AssayInsulin AntibodiesWater-Electrolyte BalanceGlycerolGlutamate DecarboxylaseNeuromyelitis OpticaMice, Inbred NZBPemphigoid, BullousMolecular Sequence DataScleroderma, SystemicPermeabilityDesmoglein 3Amino Acid SequenceImmunoglobulin MSjogren's SyndromeEpitopesRheumatoid FactorLupus VulgarisCell Membrane PermeabilityAnemia, Hemolytic, AutoimmuneLupus NephritisRNA, Small CytoplasmicsnRNP Core ProteinsPlant TranspirationThyroglobulinKidney Tubules, CollectingMyositisKidney Concentrating AbilityNon-Fibrillar CollagensReceptor-Like Protein Tyrosine Phosphatases, Class 8Diabetes Mellitus, Type 1B-LymphocytesMice, Inbred MRL lprThyroiditis, AutoimmuneDesmoglein 1Diabetes Insipidus, NephrogenicFluorescent Antibody TechniquePlant ProteinsFluorescent Antibody Technique, IndirectIon ChannelsPolyuriaImmunoblottingBiological TransportRibonucleoproteinsDermatomyositisAntibodies, Antineutrophil CytoplasmicEpitope MappingParaneoplastic SyndromesCoombs TestMembrane ProteinsIodide PeroxidaseBlotting, WesternAntigen-Antibody ComplexBinding Sites, AntibodyXenopus laevisOsmotic PressureArthritis, RheumatoidAntigen-Antibody ReactionsAntibodies, MonoclonalCross ReactionsOocytesAntidiuretic AgentsImmunohistochemistryGraves DiseaseAntibodies, Anti-IdiotypicImmunoglobulin IdiotypesRibonucleoproteins, Small NuclearCell MembranePolyendocrinopathies, AutoimmuneDisease Models, AnimalMice, Inbred C57BLKidneyTulipaVapor PressureSkin Diseases, VesiculobullousAntibodies, AnticardiolipinStiff-Person SyndromePolymyositisEye Proteins
AntibodiesMyelinOptic neuritisAutoimmuneImmunoglobulinNMOSDNeuromyelitis optica spectrum disordersClinicalTransversePathogenic autoantibodiesEnzyme-linked immunosorbent assaysAssayAnti-AQP4Assays is essentialAstrocyteHumansPlasmablastsNeuronalProteinSpectrumDiagnosticMOGADInflammatoryAntigensSeropositivePatientsPrevalenceCentral nervouNeurologyDiseasesSymptomsWaterImmuneOpticaResearchPlasmaQuantitativeRoleChildhoodFormation
Antibodies5
  • The organization of aquaporin -4 (AQP4) into large plasma membrane assemblies provides an opportunity to image membrane -bound AQP4 antibodies (AQP4- IgG ) and evaluate changes in their spatial distribution due to alterations in AQP4 isoform expression and AQP4- IgG epitope specificity . (bvsalud.org)
  • Background The presence of antibodies to aquaporin-4 (AQP4) has been identified as a key characteristic of neuromyelitis optica spectrum disorder (NMOSD), an autoimmune inflammatory demyelinating central nervous system (CNS) disorder. (guthyjacksonfoundation.org)
  • SjS is often associated with polyclonal B cell activation resulting in the presence of multiple autoantibodies including the well-known SSA and SSB antibodies. (biomedcentral.com)
  • He introduced a novel approach to treat neuromyelitis optica (NMO) by monoclonal anti- aquaporin-4 antibodies that selectively block NMO autoantibody binding to aquaporin-4. (keio.ac.jp)
  • The anti-mog spectrum in children is equally variated: Out of a sample of 41 children with MOG-antibodies 29 had clinical NMOSD (17 relapsing), 8 had ADEM (4 relapsing with ADEM-ON), 3 had a single clinical event CIS, and 1 had a relapsing tumefactive disorder. (mdwiki.org)
Myelin7
  • Background Autoantibodies to glial, myelin and neuronal antigens have been reported in a range of central demyelination syndromes and autoimmune encephalopathies in children, but there has not been a systematic evaluation across the range of central nervous system (CNS) autoantibodies in childhood-acquired demyelinating syndromes (ADS). (bmj.com)
  • For example, aquaporin-4 (AQP4) immunoglobulin G (IgG) serum autoantibody and myelin oligodendrocyte glycoprotein IgG (MOG IgG) autoantibody are specific for neuromyelitis optica spectrum disorders (NMOSDs) and MOG-associated disease (MOGAD), respectively. (arupconsult.com)
  • The clinical implications of myelin oligodendrocyte glycoprotein autoantibodies (MOG-Abs) are increasing. (annlabmed.org)
  • Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-Abs) such as AQP4-Abs are clinically significant in patients with CNS inflammatory disorders. (annlabmed.org)
  • Autoantibodies such as Aquaporin-4 autoantibodies and Myelin oligodendrocyte glycoprotein autoantibodies have been associated with transverse myelitis. (jwmdc.com)
  • Further workup demonstrated the presence of Myelin oligodendrocyte glycoprotein autoantibodies. (jwmdc.com)
  • The overlapping clinical features of relapsing remitting multiple sclerosis (RRMS), aquaporin-4 (AQP4)-antibody (Ab) neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-Ab disease mean that detection of disease specific serum antibodies is the gold standard in diagnostics. (biomedcentral.com)
Optic neuritis2
  • Abstract Neuromyelitis optica is an autoimmune disorder characterized by optic neuritis, transverse myelitis, and aquaporin-4 autoantibodies. (guthyjacksonfoundation.org)
  • MOGAD is typically associated with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), and transverse myelitis (TM) and is less commonly associated with cerebral cortical encephalitis, brainstem or cerebellar symptoms, and clinical presentations including the combination of several phenotypes and sometimes accompanies other autoantibodies such as anti- N -methy-D aspartate receptor (NMDAR) autoantibodies with symptoms of autoimmune encephalitis. (encyclopedia.pub)
Autoimmune4
  • Currently there are no serological tests for rapidly detecting autoantibodies associated with autoimmune diseases that also satisfy the growing demand for high analytical sensitivity and reproducibility. (biomedcentral.com)
  • NMO's etiology is unknown but NMO is believed to be an autoimmune disease associated with autoantibodies to aquaporin-4. (collinsquest.org)
  • Neuromyelitis optica (NMO) is an autoimmune demyelinating disease of the central nervous system in which binding of anti-aquaporin-4 (AQP4) autoantibodies (NMO-IgG) to astrocytes causes complement-dependent cytotoxicity (CDC) and inflammation resulting in oligodendrocyte and neuronal injury. (chaen-rcah.ca)
  • In fact, MOG was initially identified as an immunodominant target for demyelinating autoantibodies in a guinea pig model of experimental autoimmune encephalomyelitis (EAE) [ 2 ] [ 3 ] . (encyclopedia.pub)
Immunoglobulin2
  • The term 'neuromyelitis optica spectrum disorders' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. (nih.gov)
  • For years, neuromyelitis optica was thought to be a variant of multiple sclerosis (MS), but in 2004, a circulating immunoglobulin autoantibody was reported in patients with neuromyeltis optica that was absent in those with MS. Within a year, the astrocyte water channel protein aquaporin-4 was identified as its target, leading to several advanced therapeutics more than a decade later. (neurologylive.com)
NMOSD7
  • UPLIZNA is the first and only anti-CD19 B-cell-depleting humanized monoclonal antibody approved by the U.S. Food and Drug Administration (FDA) and European Commission (EC) for the treatment of adult patients with anti-aquaporin-4 (AQP4) antibody positive NMOSD. (minstercommunitypost.com)
  • UPLIZNA is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive. (minstercommunitypost.com)
  • Neuromyelitis optica spectrum disorders (NMOSD) are an inflammation of the central nervous system associated with autoantibodies to aquaporin-4. (ox.ac.uk)
  • This decision from the Japanese Ministry of Health, Labour and Welfare covers all NMOSD adults, with or without aquaporin-4 water channel autoantibodies (AQP4-IgG). (neuromyelitisnews.com)
  • In the case of NMOSD, however, B-cells produce "autoantibodies" that zero in on the body's own cells - in this case, nervous system support cells called glia. (neuromyelitisnews.com)
  • Uplizna is designed to reduce the risk of NMOSD attacks by binding to B-cells and eliminating them, thereby reducing autoantibody production. (neuromyelitisnews.com)
  • Neuromyelitis optica spectrum disorder (NMOSD) was previously regarded as a subtype of MS, but is now considered an independent disease, since the majority of the cases are characterized by the presence of anti-aquaporin 4 autoantibody. (go.jp)
Neuromyelitis optica spectrum disorders1
  • Neuromyelitis optica and neuromyelitis optica spectrum disorders have been recently associated with the disease-specific autoantibody aquaporin-4, thought to be pathogenic. (elsevierpure.com)
Clinical5
  • 15 The key role of aberrant B cell autoreactivity in SLE was revealed in a landmark murine study using a knockout gene mutation to prevent lupus mice from developing B cells, which resulted in a lack of autoantibody formation and clinical manifestations (nephritis or vasculitis). (bmj.com)
  • Testing for these autoantibodies is recommended in patients who present with acute central nervous system (CNS) demyelination and clinical, imaging, or laboratory features that are atypical for MS. Research to identify MS-specific autoantibodies is ongoing. (arupconsult.com)
  • We investigated clinical outcomes and prognostic characteristics of 106 aquaporin-4 antibody-seropositive patients from the UK and Japan. (elsevierpure.com)
  • Thus, age at disease onset and genetic factors are both likely to be important in determining clinical outcomes in aquaporin-4 disease. (elsevierpure.com)
  • They are sometimes considered different diseases from Multiple Sclerosis, [4] [5] but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course. (mdwiki.org)
Transverse1
  • 4 , 5 , 7 The estimated frequency of CNS involvement ranges from 10-60% in different reports, depending on the parameters studied (eg, patient selection, diagnostic criteria, etc). 4 ⇓ - 6 Patients with pSS can present with a wide range of focal or diffuse neurologic or psychiatric manifestations, including motor/sensory deficits, transverse myelitis, and cognitive impairment. (ajnr.org)
Pathogenic autoantibodies2
  • The cell-based method is time consuming but has many advantages and may provide a gold standard for the future in the detection of pathogenic autoantibodies in patients with immune-mediated diseases. (ox.ac.uk)
  • This important finding that the pathogenic autoantibodies recognize a conformational epitope on the native antigen protein led to the designation of the human anti-MOG autoantibody-associated disease [ 5 ] [ 6 ] . (encyclopedia.pub)
Enzyme-linked immunosorbent assays1
  • Over the years, extensive studies conducted in patients with MS have investigated the presence of anti-MOG autoantibodies using Western blotting and enzyme-linked immunosorbent assays targeting recombinant mouse MOG, without clear relation and specificity with MS [ 4 ] . (encyclopedia.pub)
Assay4
  • Description: A sandwich quantitative ELISA assay kit for detection of Mouse Aquaporin 4 (AQP4) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids. (fslregister.nl)
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Mouse Aquaporin 4 (AQP4) in serum, plasma, tissue homogenates, cell lysates, cell culture supernates and other biological fluids. (fslregister.nl)
  • Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Mouse Aquaporin 4 (AQP4) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates and other biological fluids with no significant corss-reactivity with analogues from other species. (fslregister.nl)
  • In recent years, the presence of anti-MOG autoantibodies has been extensively tested in patients with CNS inflammatory diseases using a cell-based assay that preserves the conformational structure of the full-length human MOG [ 7 ] . (encyclopedia.pub)
Anti-AQP42
Assays is essential1
Astrocyte2
  • In the CNS, AQP4 is the most prevalent aquaporin channel, specifically located at the perimicrovessel astrocyte foot processes, glia limitans, and ependyma. (wikipedia.org)
  • Other performances that aquaporin-4 is involved in are synaptic plasticity, astrocyte migration, regulation of extracellular space volume, and the homeostasis of potassium. (wikipedia.org)
Humans1
  • Aquaporin-4, also known as AQP-4, is a water channel protein encoded by the AQP4 gene in humans. (wikipedia.org)
Plasmablasts1
Neuronal1
  • Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. (jamanetwork.com)
Protein2
  • The target antigen of NMO-IgG was recently identified as aquaporin-4 (AQP4) water channel protein, which is mainly expressed in brain and spinal cord. (go.jp)
  • developed transgenic mice bearing MOG peptide-specific T cell receptors, resulting in spontaneous relapsing-remitting EAE along with the expansion of autoreactive B cells that produce autoantibodies binding to a conformational epitope on the native MOG protein [ 3 ] . (encyclopedia.pub)
Spectrum2
Diagnostic1
  • The International Consensus Group on MOG autoantibody-associated disease (MOGAD) has proposed that the diagnostic criteria for MOGAD should include the presence of anti-MOG autoantibodies detected using cell-based assays. (encyclopedia.pub)
MOGAD1
  • The majority of adult patients who are positive for anti-MOG autoantibodies exhibit ON or TM, while ADEM with or without ON is the most frequent presentation in pediatric patients with MOGAD. (encyclopedia.pub)
Inflammatory1
  • Inflammation in lupus is caused by the formation of autoantibodies and immune complexes and can cause inflammatory responses and activate the complement system. (bmj.com)
Antigens2
  • Using QLIPS, autoantibodies to three SjS antigens, La, Ro60, and Ro52 were detected in 49%, 56% and 70%, respectively, of the SjS patients and none of the controls (100% specificity). (biomedcentral.com)
  • Within a framework of the gut-brain axis, Dr. Severance will review the role of inflammation, infectious agents, food antigens, gut dysbioses, endothelial barrier instabilities and autoantibody propagation on CNS pathologies such as neurotransmitter receptor hypofunction and complement pathway-mediated synaptic pruning. (neuroimmune.org)
Seropositive1
  • Lucchinetti CF, Kimmel DW, Lennon VA. Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. (jamanetwork.com)
Patients7
  • 4 ⇓ - 6 The current data from MRI studies support an increased frequency of high signal intensity lesions in the periventricular and/or subcortical WM on FLAIR and T2-weighted imaging, observed mainly in patients with pSS and evidence of CNS disease. (ajnr.org)
  • What is the role of autoantibody testing in patients with multiple sclerosis? (arupconsult.com)
  • Together these results suggest that the QLIPS format for Ro52 yields both a more rapid and more discriminating test for detecting Ro52 autoantibodies than existing immunoassays and has the potential to be adapted for point-of-care evaluation of patients with SjS and other rheumatologic diseases. (biomedcentral.com)
  • Anti-MOG autoantibodies have been detected in many EAE variants, inciting many promising studies in patients with CNS demyelinating diseases. (encyclopedia.pub)
  • Progression to wheelchair dependence was faster among patients with Purkinje cell cytoplasmic antibody type 1 (PCA-1) seropositivity and glutamic acid decarboxylase 65-kDa autoantibody (GAD65) seronegativity. (jamanetwork.com)
  • Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late-onset insulin-dependent diabetes mellitus, and polyendocrine autoimmunity. (jamanetwork.com)
  • Some NMO patients present double positive for autoantibodies to AQP4 and MOG. (mdwiki.org)
Prevalence3
  • We aimed to validate the prevalence of anti-aquaporin 5 (AQP5) IgG in a non-Korean cohort and to optimize the method to screen the anti-AQP5 IgG. (mdpi.com)
  • 4 ⇓ - 6 Although involvement of the peripheral nervous system is a well-documented feature of the disease, the prevalence, the type, and the underlying mechanism of CNS involvement remain unclear. (ajnr.org)
  • 4-6 The prevalence of SLE is different between age, gender, geographic and racial distributions. (bmj.com)
Central nervou2
  • A limited number of aquaporins are found within the central nervous system (CNS): AQP1, 3, 4, 5, 8, 9, and 11, but more exclusive representation of AQP1, 4, and 9 are found in the brain and spinal cord. (wikipedia.org)
  • Aquaporin-4's overall function is to provide fast water transportation as well as maintain homeostatic balance within the central nervous system. (wikipedia.org)
Neurology1
  • 4 Department of Neurology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. (nih.gov)
Diseases3
  • Autoantibody testing is important to differentiate between multiple sclerosis (MS) and other similar diseases. (arupconsult.com)
  • One major approach involves using antibody-based tests to diagnose and even predict the onset of various diseases [ 1 , 4 ]. (biomedcentral.com)
  • He also presented that aquaporin-4 knockout had an effect on symptoms of neurodegenerative diseases. (keio.ac.jp)
Symptoms1
Water2
Immune1
  • The autoantibodies trigger an immune reaction upon binding to these supportive cells, damaging them and their surrounding nerve cells through the prolonged inflammation that ensues. (neuromyelitisnews.com)
Optica1
Research1
  • In Keynote Lecture 4, Professor Masato Yasui of the Department of Pharmacology at the Keio University School of Medicine presented his research on aquaporin-4. (keio.ac.jp)
Plasma1
  • The aquaporin-4 tetramers accumulate to transform into orthogonal arrays of particle (OAPs) in the cell plasma membrane. (wikipedia.org)
Quantitative1
  • Here we investigated whether a quick version of luciferase immunoprecipitation systems (QLIPS) could be used to produce a rapid, specific and quantitative test to detect autoantibodies associated with SjS. (biomedcentral.com)
Role1
  • Within the retina, the role of AQP-4 is to maintain homeostasis. (wikipedia.org)
Childhood1
  • Conclusions A range of CNS-directed autoantibodies were found in association with childhood ADS. (bmj.com)
Formation1
  • Aquaporin-4 is essential in the formation of memory as well as synaptic plasticity. (wikipedia.org)