Cargando…

Distinctive accumulation of globotriaosylceramide and globotriaosylsphingosine in a mouse model of classic Fabry disease

Fabry disease (FD) is an inherited disease caused by deficient α-galactosidase A activity that is characterized by the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3). Although plasma lyso-Gb3 is a sensitive biomarker of FD, the correlation between its concentrati...

Descripción completa

Detalles Bibliográficos
Autores principales:	Taguchi, Atsumi, Ishii, Satoshi, Mikame, Mariko, Maruyama, Hiroki
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Elsevier 2023
Materias:	Research Paper
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9823212/ https://www.ncbi.nlm.nih.gov/pubmed/36624895 http://dx.doi.org/10.1016/j.ymgmr.2022.100952

Ejemplares similares

Does administration of hydroxychloroquine/amiodarone accelerate accumulation of globotriaosylceramide and globotriaosylsphingosine in Fabry mice?
por: Tsukimura, Takahiro, et al.
Publicado: (2021)

Determination of globotriaosylceramide analogs in the organs of a mouse model of Fabry disease
por: Ishii, Satoshi, et al.
Publicado: (2020)

Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an α-Galactosidase A Knockout Mouse, a Model of Fabry Disease
por: Sueoka, Hideaki, et al.
Publicado: (2015)

Plasma Globotriaosylsphingosine and α-Galactosidase A Activity as a Combined Screening Biomarker for Fabry Disease in a Large Japanese Cohort
por: Maruyama, Hiroki, et al.
Publicado: (2021)

Epithelial–Mesenchymal Transition in Kidney Tubular Epithelial Cells Induced by Globotriaosylsphingosine and Globotriaosylceramide
por: Jeon, Yeo Jin, et al.
Publicado: (2015)

Low bone mineral density due to secondary hyperparathyroidism in the Gla(tm)Tg(CAG‐A4GALT) mouse model of Fabry disease
por: Maruyama, Hiroki, et al.
Publicado: (2020)

Medullary thick ascending limb impairment in the Gla(tm)Tg(CAG-A4GALT) Fabry model mice
por: Maruyama, Hiroki, et al.
Publicado: (2018)

A new mutation found in newborn screening for Fabry disease evaluated by plasma globotriaosylsphingosine levels
por: Chinen, Yasutsugu, et al.
Publicado: (2017)

Globotriaosylsphingosine Accumulation and Not Alpha-Galactosidase-A Deficiency Causes Endothelial Dysfunction in Fabry Disease
por: Namdar, Mehdi, et al.
Publicado: (2012)

Plasma mutant α-galactosidase A protein and globotriaosylsphingosine level in Fabry disease
por: Tsukimura, Takahiro, et al.
Publicado: (2014)

Monitoring globotriaosylsphingosine in a Korean male patient with Fabry disease
por: Jang, Heejoon, et al.
Publicado: (2019)

Correction: Globotriaosylceramide Accumulation and Not Alpha-Galactosidase-A Deficiency Causes Endothelial Dysfunction in Fabry Disease
por: Namdar, Mehdi, et al.
Publicado: (2012)

Skin globotriaosylceramide 3 deposits are specific to Fabry disease with classical mutations and associated with small fibre neuropathy
por: Liguori, Rocco, et al.
Publicado: (2017)

Clinical significance of plasma globotriaosylsphingosine levels in Chinese patients with Fabry disease
por: Ouyang, Yan, et al.
Publicado: (2018)

Screening for Fabry Disease by Urinary Globotriaosylceramide Isoforms Measurement in Patients with Left Ventricular Hypertrophy
por: Gaggl, Martina, et al.
Publicado: (2016)

Migalastat HCl Reduces Globotriaosylsphingosine (Lyso-Gb(3)) in Fabry Transgenic Mice and in the Plasma of Fabry Patients
por: Young-Gqamana, Brandy, et al.
Publicado: (2013)

Skin Globotriaosylceramide 3 Load Is Increased in Men with Advanced Fabry Disease
por: Üçeyler, Nurcan, et al.
Publicado: (2016)

A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse
por: Shin, Seok-Ho, et al.
Publicado: (2018)

Spontaneous Accumulation of Globotriaosylceramide (Gb3) in Proximal Renal Tubules in an ICR Mouse
por: Mutsuga, Mayu, et al.
Publicado: (2013)

Pulmonary involvement in Fabry disease: effect of plasma globotriaosylsphingosine and time to initiation of enzyme replacement therapy
por: Franzen, Daniel, et al.
Publicado: (2018)

Glycosphingolipid storage in Fabry mice extends beyond globotriaosylceramide and is affected by ABCB1 depletion
por: Kamani, Mustafa A, et al.
Publicado: (2016)

Ceria-Zirconia nanoparticles reduce intracellular globotriaosylceramide accumulation and attenuate kidney injury by enhancing the autophagy flux in cellular and animal models of Fabry disease
por: An, Jong Hun, et al.
Publicado: (2022)

Comparative urinary globotriaosylceramide analysis by thin-layer chromatography-immunostaining and liquid chromatography-tandem mass spectrometry in patients with Fabry disease
por: Shiga, Tomoko, et al.
Publicado: (2021)

Serum Globotriaosylceramide Assay as a Screening Test for Fabry Disease in Patients with ESRD on Maintenance Dialysis in Korea
por: Kim, Jeong-Yup, et al.
Publicado: (2010)

A Novel Rapid MALDI-TOF-MS-Based Method for Measuring Urinary Globotriaosylceramide in Fabry Patients
por: Alharbi, Fahad J., et al.
Publicado: (2016)

One Year of Enzyme Replacement Therapy Reduces Globotriaosylceramide Inclusions in Podocytes in Male Adult Patients with Fabry Disease
por: Najafian, Behzad, et al.
Publicado: (2016)

Role of Globotriaosylceramide in Physiology and Pathology
por: Celi, Ana Beatriz, et al.
Publicado: (2022)

Analysis of Lyso-Globotriaosylsphingosine in Dried Blood Spots
por: Johnson, Britt, et al.
Publicado: (2013)

Inhibition of tumor angiogenesis by globotriaosylceramide immunotargeting
por: Birklé, Stéphane, et al.
Publicado: (2013)

Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types
por: Welford, R W D, et al.
Publicado: (2018)

Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis
por: Maruyama, Hiroki, et al.
Publicado: (2018)

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis
por: Maruyama, Hiroki, et al.
Publicado: (2019)

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis
por: Maruyama, Hiroki, et al.
Publicado: (2018)

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis
por: Maruyama, Hiroki, et al.
Publicado: (2018)

Massive accumulation of globotriaosylceramide in various tissues from a Fabry patient with a high antibody titer against alpha-galactosidase A after 6 years of enzyme replacement therapy
por: Hongo, Kenichi, et al.
Publicado: (2020)

Reduction of podocyte globotriaosylceramide content in adult male patients with Fabry disease with amenable GLA mutations following 6 months of migalastat treatment
por: Mauer, Michael, et al.
Publicado: (2017)

MiRNA Let-7a and Let-7d Are Induced by Globotriaosylceramide via NF-kB Activation in Fabry Disease
por: Maier, Nadine, et al.
Publicado: (2021)

A new multiplex analysis of glucosylsphingosine and globotriaosylsphingosine in dried blood spots by tandem mass spectrometry
por: Van Baelen, Amber, et al.
Publicado: (2023)

Mass Spectrometry Analysis of Globotriaosylsphingosine and Its Analogues in Dried Blood Spots
por: Boutin, Michel, et al.
Publicado: (2023)

Renal globotriaosylceramide deposits for Fabry disease linked to uncertain pathogenicity gene variant c.352C>T/p.Arg118Cys: A family study
por: Cerón‐Rodríguez, Magdalena, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_jkq772vaq8jgo13h4k8tum13pv