Database : MEDLINE
Search on : Gangliosidoses [Words]
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[PMID]: 29358305
[Au] Autor:Allende ML; Cook EK; Larman BC; Nugent A; Brady JM; Golebiowski D; Sena-Esteves M; Tifft CJ; Proia RL
[Ad] Address:Genetics of Development and Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892.
[Ti] Title:Cerebral organoids derived from Sandhoff disease-induced pluripotent stem cells exhibit impaired neurodifferentiation.
[So] Source:J Lipid Res;59(3):550-563, 2018 Mar.
[Is] ISSN:1539-7262
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Sandhoff disease, one of the GM2 gangliosidoses, is a lysosomal storage disorder characterized by the absence of ß-hexosaminidase A and B activity and the concomitant lysosomal accumulation of its substrate, GM2 ganglioside. It features catastrophic neurodegeneration and death in early childhood. How the lysosomal accumulation of ganglioside might affect the early development of the nervous system is not understood. Recently, cerebral organoids derived from induced pluripotent stem (iPS) cells have illuminated early developmental events altered by disease processes. To develop an early neurodevelopmental model of Sandhoff disease, we first generated iPS cells from the fibroblasts of an infantile Sandhoff disease patient, then corrected one of the mutant alleles in those iPS cells using CRISPR/Cas9 genome-editing technology, thereby creating isogenic controls. Next, we used the parental Sandhoff disease iPS cells and isogenic -corrected iPS cell clones to generate cerebral organoids that modeled the first trimester of neurodevelopment. The Sandhoff disease organoids, but not the -corrected organoids, accumulated GM2 ganglioside and exhibited increased size and cellular proliferation compared with the -corrected organoids. Whole-transcriptome analysis demonstrated that development was impaired in the Sandhoff disease organoids, suggesting that alterations in neuronal differentiation may occur during early development in the GM2 gangliosidoses.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:In-Data-Review
[do] DOI:10.1194/jlr.M081323

  2 / 2200 MEDLINE  
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[PMID]: 29352662
[Au] Autor:Nestrasil I; Ahmed A; Utz JM; Rudser K; Whitley CB; Jarnes-Utz JR
[Ad] Address:Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA.
[Ti] Title:Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study.
[So] Source:Mol Genet Metab;123(2):97-104, 2018 Feb.
[Is] ISSN:1096-7206
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: GM1-gangliosidosis and GM2-gangliosidosis (Tay-Sachs disease and Sandhoff disease) are unrelenting heritable neurodegenerative conditions of lysosomal ganglioside accumulation. Although progressive brain atrophy is characteristic, longitudinal quantification of specific brain structures has not been systematically studied. OBJECTIVES: The goal of this longitudinal study has been to quantify and track brain MRI volume changes, including specific structure volume changes, at different times in disease progression of childhood gangliosidoses, and to explore quantitative brain MRI volumetry (qMRI) as a non-invasive marker of disease progression for future treatment trials. METHODS: Brain qMRI studies were performed in 14 patients with gangliosidoses (9 infantile, 5 juvenile) yearly. Cerebellar cortex and white matter, caudate, putamen, corpus callosum, ventricles, total brain, and intracranial volumes were measured, as well as total brain volume. Age-matched controls were available for the patients with the juvenile phenotype. RESULTS: The infantile phenotype of all gangliosidoses showed a consistent pattern of macrocephaly and rapidly increasing intracranial MRI volume with both (a) brain tissue volume (cerebral cortex and other smaller structures) and (b) ventricular volume (P<0.01 for all). In contrast to apparent enlargement of the total brain volume, and chiefly the enlarged cerebral cortex, a subset of smaller brain substructures generally decreased in size: the corpus callosum, caudate and putamen became smaller with time. The volume of cerebellar cortex also decreased in patients with infantile GM1-gangliosidosis and juvenile GM1- and GM2-gangliosidosis; however, infantile GM2-gangliosidosis cerebellar cortex was the exception, increasing in size. Elevated intracranial pressure (estimated by lumbar spinal pressure) was a common finding in infantile disease and showed continued increases as the disease progressed, yet lacked MRI signs of hydrocephalus except for increasing ventricular size. Notably, in patients with juvenile gangliosidosis, macrocephaly and elevated intracranial pressure were absent and total brain volume decreased with time compared to controls (P=0.004). CONCLUSIONS: The disease course of infantile versus juvenile gangliosidoses is clearly distinguished by the rate of brain disease progression as characterized by qMRI. Assessments by qMRI represent a robust non-invasive method for monitoring CNS changes in the clinical course of gangliosidoses and is ideally suited to monitor effects of novel CNS-directed therapies in future clinical trials.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180302
[Lr] Last revision date:180302
[St] Status:In-Data-Review

  3 / 2200 MEDLINE  
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[PMID]: 29227082
[Au] Autor:Olkhovych NV
[Ti] Title:Chitotriosidase activity as additional biomarker in the diagnosis of lysosomal storage diseases.
[So] Source:Ukr Biochem J;88(1):69-78, 2016 Jan-Feb.
[Is] ISSN:2409-4943
[Cp] Country of publication:Ukraine
[La] Language:eng
[Ab] Abstract:To date, several genetic variants that lead to a deficiency of chitotriosidase activity have been described. The duplication of 24 bp (dup24bp) in exon 10 of the CHIT1 gene, which causes a complete loss of enzymatic activity of the gene product, is the most common among the European population. The aim of the study was to evaluate the possibility of using chitotriosidase activity as an additional biomarker in diagnosis of lysosomal storage diseases (LSDs) in Ukraine, to determine this parameter in blood plasma of the patients with various lysosomal diseases and to assess the effect of the presence of dup24bp in the CHIT1 gene on this parameter. It has been shown that chitotriosidase activity in blood plasma is a convenient additional biochemical marker in the diagnosis of some LSDs, namely Gaucher disease, Niemann-Pick disease A, B, C and GM1-gangliosidosis. Reference ranges of the normal chitotriosidase activity were determined in blood plasma of Ukrainian population and found to be 8.0-53.1 nmol 4-methylumbelliferone/h·ml of plasma. The total allele frequency of the dup24bp in the CHIT1 gene in Ukrainian population was determined, which amounted to 0.26 (323/1244) that is higher than in European population. It was indicated that moleculargenetic screening of dup24bp in the CHIT1 gene is a necessary stage in a protocol for the laboratory diagnosis of Gaucher disease, Niemann-Pick disease A, B, C as well as GM1-gangliosidosis to avoid incorrect diagnosis.
[Mh] MeSH terms primary: Gangliosidosis, GM1/genetics
Gaucher Disease/genetics
Gene Frequency
Hexosaminidases/genetics
Niemann-Pick Diseases/genetics
[Mh] MeSH terms secundary: Adult
Alleles
Biomarkers/metabolism
Case-Control Studies
Exons
Female
Gangliosidosis, GM1/classification
Gangliosidosis, GM1/diagnosis
Gangliosidosis, GM1/pathology
Gaucher Disease/diagnosis
Gaucher Disease/pathology
Gene Duplication
Gene Expression
Genetic Testing
Hexosaminidases/blood
Hexosaminidases/deficiency
Humans
Hymecromone/blood
Male
Niemann-Pick Diseases/classification
Niemann-Pick Diseases/diagnosis
Niemann-Pick Diseases/pathology
Ukraine
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Biomarkers); 3T5NG4Q468 (Hymecromone); EC 3.2.1.- (Hexosaminidases); EC 3.2.1.- (chitotriosidase)
[Em] Entry month:1801
[Cu] Class update date: 180116
[Lr] Last revision date:180116
[Js] Journal subset:IM
[Da] Date of entry for processing:171212
[St] Status:MEDLINE
[do] DOI:10.15407/ubj88.01.069

  4 / 2200 MEDLINE  
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[PMID]: 28748763
[Au] Autor:Nair M; Sandhu SS; Sharma AK
[Ad] Address:Stem Cell Technology Laboratory, Centre for Scientific Research & Development, People's University, Bhopal, M.P. - 462037. India.
[Ti] Title:Induced Pluripotent Stem Cell Technology: A Paradigm Shift in Medical Science for Drug Screening and Disease Modeling.
[So] Source:Curr Med Chem;24(39):4368-4398, 2017.
[Is] ISSN:1875-533X
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:BACKGROUND: Induced Pluripotent Stem Cell (IPSC) Technology is the most advanced research as it offers an attractive alternative for establishing patient-specific IPSCs to recapitulate phenotypes of not only monogenic diseases (viz. Thalassaemia, Sickle cell anemia, Haemophilia, Tay-Sachs disease), but also late-onset polygenic diseases (viz. Parkinson's disease, Alzheimer's disease, schizophrenia). Over the hindsight, numerous studies of the past and current scientists have led to the production, maturation and understanding of induced pluripotent stem cell technology and its use in basic and clinical research. METHODS: A systematic search of peer-reviewed scientific literature and clinical trials in public databases were carried out to summarize the evidence on the use of IPSC. RESULTS: Current review sheds light upon the use of patient-derived iPSC models in drug toxicity, screening and discovery which have been derived after referring to more than 200 articles in literature. Furthermore, their use as disease models was also studied signifying the versatility of iPSC lines. CONCLUSION: Through this review, we describe the advent of iPSC technology, where we comprehensively cover the generation of iPSCs and their characterization along with their prospective applications using IPSC banks in disease modeling and drug screening.
[Mh] MeSH terms primary: Disease Models, Animal
Induced Pluripotent Stem Cells
Stem Cell Transplantation
[Mh] MeSH terms secundary: Alzheimer Disease/therapy
Anemia, Sickle Cell/therapy
Animals
Drug Evaluation, Preclinical
Hemophilia A/therapy
Humans
Parkinson Disease/therapy
Schizophrenia/therapy
Tay-Sachs Disease/therapy
Thalassemia/therapy
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1801
[Cu] Class update date: 180101
[Lr] Last revision date:180101
[Js] Journal subset:IM
[Da] Date of entry for processing:170728
[St] Status:MEDLINE
[do] DOI:10.2174/0929867324666170727100508

  5 / 2200 MEDLINE  
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[PMID]: 28923328
[Au] Autor:Duarte AJ; Ribeiro D; Oliveira P; Amaral O
[Ad] Address:Departamento de Genética Humana-Unidade I and D-P, CSPGF, Instituto Nacional de Saúde Ricardo Jorge (INSA, IP), Porto, Portugal.
[Ti] Title:Mutation Frequency of Three Neurodegenerative Lysosomal Storage Diseases: From Screening to Treatment?
[So] Source:Arch Med Res;48(3):263-269, 2017 Apr.
[Is] ISSN:1873-5487
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: The ascertainment of mutation frequencies in the general population may have impact on the population's wellbeing and respective healthcare services. Furthermore, it may help define which approaches will be more effective for certain patients based on the genetic cause of disease. AIM OF THE STUDY: Determine the frequency of three mutations, known to be a major cause of three distinct Lysosomal Storage Diseases (LSDs). METHODS: The following pre-requisites were met: each mutation accounted for over 55% of the disease alleles among previously reported unrelated patients, all three diseases were among the most prevalent LSDs in the population under study, they all involved devastating deterioration of the nervous system, lacked curative treatment and may be fatal in childhood or adolescence. The anonymous samples used in this study were representative of the whole population; mutations were tested by PCR based methods, positive results were further confirmed. The diseases studied were Mucopolysaccharidosis type I (Hurler, MIM 607014), Tay Sachs disease variant B1 (TS, MIM 272800) and Metachromatic Leukodystrophy (MLD, MIM 250100); the mutations were, respectively, p.W402X, p.R178C and c.465+1G>A. RESULTS AND CONCLUSION: Increased carrier frequencies were found for Tay Sachs disease variant B1 HEXA p.R178C mutation (1:340) and for the infantile MLD ARSA c.465+1G> A mutation (1:350) denoting higher risk for these sub-types of disease in Portugal and possibly in individuals of Iberian ancestry. Carrier screening in target populations may provide the foundations for more effective approaches to precision medicine.
[Mh] MeSH terms primary: Leukodystrophy, Metachromatic/genetics
Mucopolysaccharidosis I/genetics
Tay-Sachs Disease/genetics
[Mh] MeSH terms secundary: Alleles
Humans
Infant, Newborn
Mutation
Mutation Rate
Portugal
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171023
[Lr] Last revision date:171023
[Js] Journal subset:IM
[Da] Date of entry for processing:170920
[St] Status:MEDLINE

  6 / 2200 MEDLINE  
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[PMID]: 28895707
[Au] Autor:Mugnaini J; Pereyra M; Dodelson de Kremer R; Gamboni B; Argaraña CE; Oller Ramírez AM
[Ad] Address:Centro de Estudio de las Metabolopatías Congénitas (CEMECO), Cátedra de Clínica Pediátrica, Facultad de Ciencias Médicas, Universidad Nacional de Córdoba (UNC), Córdoba, Argentina. juliamugnaini@gmail.com.
[Ti] Title:Variante juvenil de la enfermedad de Sandhoff: presentación del primer caso descrito en Argentina. [Juvenile form of Sandhoff disease: first case reported in Argentina].
[So] Source:Arch Argent Pediatr;115(5):e298-e301, 2017 Oct 01.
[Is] ISSN:1668-3501
[Cp] Country of publication:Argentina
[La] Language:spa
[Ab] Abstract:Sandhoff disease is a neurodegenerative, lysosomal and autosomal recessive disease caused by mutations in the HEXB gene. Three forms are recognized: infantile, juvenile and adult. Previously, an endogamous population in Córdoba, Argentina, was identified with a high incidence of Sandhoff disease, all reported cases were of the infantile type. In this work, we describe a child with the juvenile form of Sandhoff disease, the first case reported in Argentina. The patient is a 7-year-old boy presenting with ataxia, speech disturbances and global developmental delay, symptoms starting at the age of 2 years. Diagnosis was based on the hexosaminidase deficiency. Sequencing of genomic DNA revealed compound heterozygosity for two HEXB gene mutations: c.796T>G (p.Y266D) and c.1615C>T (p.R539C), both already reported.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 170912
[Lr] Last revision date:170912
[St] Status:In-Process
[do] DOI:10.5546/aap.2017.e298

  7 / 2200 MEDLINE  
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[PMID]: 28879940
[Au] Autor:Kamate M; Mittal N
[Ad] Address:Department of Paediatrics, KLE University's J N Medical College, Belgaum, Karnataka, India.
[Ti] Title:Isolated globus pallidi hypointensities in type 2 GM1 gangliosidoses.
[So] Source:Neurol India;65(5):1195-1196, 2017 Sep-Oct.
[Is] ISSN:0028-3886
[Cp] Country of publication:India
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 170907
[Lr] Last revision date:170907
[St] Status:In-Data-Review
[do] DOI:10.4103/neuroindia.NI_926_16

  8 / 2200 MEDLINE  
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[PMID]: 28716012
[Au] Autor:Karimzadeh P; Naderi S; Modarresi F; Dastsooz H; Nemati H; Farokhashtiani T; Shamsian BS; Inaloo S; Faghihi MA
[Ad] Address:Pediatric Neurology Department, Pediatric Neurology Research Center, Shahid Beheshti University of Medical Sciences (SBMU), Tehran, Iran.
[Ti] Title:Case reports of juvenile GM1 gangliosidosisis type II caused by mutation in GLB1 gene.
[So] Source:BMC Med Genet;18(1):73, 2017 Jul 17.
[Is] ISSN:1471-2350
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Type II or juvenile GM1-gangliosidosis is an autosomal recessive lysosomal storage disorder, which is clinically distinct from infantile form of the disease by the lack of characteristic cherry-red spot and hepatosplenomegaly. The disease is characterized by slowly progressive neurodegeneration and mild skeletal changes. Due to the later age of onset and uncharacteristic presentation, diagnosis is frequently puzzled with other ataxic and purely neurological disorders. Up to now, 3-4 types of GM1-gangliosidosis have been reported and among them type I is the most common phenotype with the age of onset around 6 months. Various forms of GM1-gangliosidosis are caused by GLB1 gene mutations but severity of the disease and age of onset are directly related to the position and the nature of deleterious mutations. However, due to its unique genetic cause and overlapping clinical features, some researchers believe that GM1 gangliosidosis represents an overlapped disease spectrum instead of four distinct types. CASE PRESENTATION: Here, we report a less frequent type of autosomal recessive GM1 gangliosidosis with perplexing clinical presentation in three families in the southwest part of Iran, who are unrelated but all from "Lurs" ethnic background. To identify disease-causing mutations, Whole Exome Sequencing (WES) utilizing next generation sequencing was performed. Four patients from three families were investigated with the age of onset around 3 years old. Clinical presentations were ataxia, gate disturbances and dystonia leading to wheelchair-dependent disability, regression of intellectual abilities, and general developmental regression. They all were born in consanguineous families with no previous documented similar disease in their parents. A homozygote missense mutation in GLB1 gene (c. 601 G > A, p.R201C) was found in all patients. Using Sanger sequencing this identified mutation was confirmed in the proband, their parents, grandparents, and extended family members, confirming its autosomal recessive pattern of inheritance. CONCLUSIONS: Our study identified a rare pathogenic missense mutation in GLB1 gene in patients with complex neurodevelopmental findings, which can extend the list of differential diagnoses for childhood ataxia in Iranian patients.
[Mh] MeSH terms primary: Gangliosidosis, GM1/genetics
Mutation, Missense
beta-Galactosidase/genetics
[Mh] MeSH terms secundary: Child
Child, Preschool
Consanguinity
Female
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:EC 3.2.1.23 (GLB1 protein, human); EC 3.2.1.23 (beta-Galactosidase)
[Em] Entry month:1707
[Cu] Class update date: 170724
[Lr] Last revision date:170724
[Js] Journal subset:IM
[Da] Date of entry for processing:170719
[St] Status:MEDLINE
[do] DOI:10.1186/s12881-017-0417-4

  9 / 2200 MEDLINE  
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[PMID]: 28687233
[Au] Autor:Rigante D; Cipolla C; Basile U; Gulli F; Savastano MC
[Ad] Address:Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy. Electronic address: drigante@gmail.com.
[Ti] Title:Overview of immune abnormalities in lysosomal storage disorders.
[So] Source:Immunol Lett;188:79-85, 2017 Aug.
[Is] ISSN:1879-0542
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:The critical relevance of the lysosomal compartment for normal cellular function can be proved by numbering the clinical phenotypes that arise in lysosomal storage disorders (LSDs), a group of around 70 different monogenic autosomal or X-linked syndromes, caused by specific lysosomal enzyme deficiencies: all LSDs are characterized by progressive accumulation of heterogeneous biologic materials in the lysosomes of various parts of the body such as viscera, skeleton, skin, heart, and central nervous system. At least a fraction of LSDs has been associated with mixed abnormalities involving the immune system, while some patients with LSDs may result more prone to autoimmune phenomena. A large production of proinflammatory cytokines has been observed in Gaucher and Fabry diseases, and wide different autoantibody production has been also reported in both. Many immune-mediated reactions are crucial to the pathogenesis of different inflammatory signs in mucopolysaccharidoses, and subverted heparan sulphate catabolism might dysregulate cellular homeostasis in the brain of these patients. Furthermore, an inappropriate activation of microglia is implicated in the neurodegenerative foci of Niemann-Pick disease, in which abnormal signalling pathways are activated by impaired sphingolipid metabolism. In addition, not the simple impaired catabolism of gangliosides per se, but also the production of anti-ganglioside autoantibodies contributes to the neurological disease of gangliosidoses. Even if the exact relationship between the modification of lysosomal activities and modulation of the immune system remains obscure, there is emerging evidence of different impaired immunity responses in a variety of LSDs: in this review we investigate and summarize the immune abnormalities and/or clinical data about immune system irregularities which have been described in a subset of LSDs.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1707
[Cu] Class update date: 170723
[Lr] Last revision date:170723
[St] Status:In-Process

  10 / 2200 MEDLINE  
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[PMID]: 28600215
[Au] Autor:Schalli M; Weber P; Tysoe C; Pabst BM; Thonhofer M; Paschke E; Stütz AE; Tschernutter M; Windischhofer W; Withers SG
[Ad] Address:Glycogroup, Institute of Organic Chemistry, Graz University of Technology, Stremayrgasse 9, A-8010 Graz, Austria.
[Ti] Title:A new type of pharmacological chaperone for G -gangliosidosis related human lysosomal ß-galactosidase: N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols.
[So] Source:Bioorg Med Chem Lett;27(15):3431-3435, 2017 08 01.
[Is] ISSN:1464-3405
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:N-Functionalized amino(hydroxymethyl)cyclopentanetriols are potent inhibitors of ß-d-galactosidases and, for the first time, could be shown to act as pharmacological chaperones for G -gangliosidosis-associated lysosomal acid ß-galactosidase thus representing a new structural type of pharmacological chaperones for this lysosomal storage disease.
[Mh] MeSH terms primary: Cyclopentanes/chemistry
Cyclopentanes/pharmacology
Enzyme Inhibitors/chemistry
Enzyme Inhibitors/pharmacology
Gangliosidosis, GM1/drug therapy
beta-Galactosidase/antagonists & inhibitors
[Mh] MeSH terms secundary: Amination
Animals
Cattle
Gangliosidosis, GM1/enzymology
Humans
Lysosomes/drug effects
Lysosomes/enzymology
Methylation
beta-Galactosidase/metabolism
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (Cyclopentanes); 0 (Enzyme Inhibitors); EC 3.2.1.23 (acid beta-galactosidase); EC 3.2.1.23 (beta-Galactosidase)
[Em] Entry month:1708
[Cu] Class update date: 171125
[Lr] Last revision date:171125
[Js] Journal subset:IM
[Da] Date of entry for processing:170611
[St] Status:MEDLINE


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