Cargando…

Thymic Alterations in GM2 Gangliosidoses Model Mice

BACKGROUND: Sandhoff disease is a lysosomal storage disorder characterized by the absence of β-hexosaminidase and storage of GM2 ganglioside and related glycolipids. We have previously found that the progressive neurologic disease induced in Hexb (−/−) mice, an animal model for Sandhoff disease, is...

Descripción completa

Detalles Bibliográficos
Autores principales:	Kanzaki, Seiichi, Yamaguchi, Akira, Yamaguchi, Kayoko, Kojima, Yoshitsugu, Suzuki, Kyoko, Koumitsu, Noriko, Nagashima, Yoji, Nagahama, Kiyotaka, Ehara, Michiko, Hirayasu, Yoshio, Ryo, Akihide, Aoki, Ichiro, Yamanaka, Shoji
Formato:	Texto
Lenguaje:	English
Publicado:	Public Library of Science 2010
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2938369/ https://www.ncbi.nlm.nih.gov/pubmed/20856892 http://dx.doi.org/10.1371/journal.pone.0012105

Ejemplares similares

The GM2 gangliosidoses: Unlocking the mysteries of pathogenesis and treatment
por: Toro, Camilo, et al.
Publicado: (2021)

The diagnostic journey for patients with late-onset GM2 Gangliosidoses
por: Lopshire, Mariah C., et al.
Publicado: (2023)

GM2 Gangliosidoses: Clinical Features, Pathophysiological Aspects, and Current Therapies
por: Leal, Andrés Felipe, et al.
Publicado: (2020)

Natural history study of glycan accumulation in large animal models of GM2 gangliosidoses
por: Cavender, Catlyn, et al.
Publicado: (2020)

L-Arginine Ameliorates Defective Autophagy in GM2 Gangliosidoses by mTOR Modulation
por: Castejón-Vega, Beatriz, et al.
Publicado: (2021)

Identification of a novel HEXB Mutation in an Iranian Family with suspected patient to GM2‐gangliosidoses
por: Mansouri‐Movahed, Fatemeh, et al.
Publicado: (2020)

In situ detection of GM1 and GM2 gangliosides using immunohistochemical and immunofluorescent techniques for auxiliary diagnosis of canine and feline gangliosidoses
por: Kohyama, Moeko, et al.
Publicado: (2016)

Combined replacement effects of human modified β-hexosaminidase B and GM2 activator protein on GM2 gangliosidoses fibroblasts
por: Kitakaze, Keisuke, et al.
Publicado: (2016)

Chitotriosidase as a biomarker for gangliosidoses
por: Kim, Sarah, et al.
Publicado: (2021)

Plasma neurofilament light, glial fibrillary acidic protein and lysosphingolipid biomarkers for pharmacodynamics and disease monitoring of GM2 and GM1 gangliosidoses patients
por: Welford, Richard W.D., et al.
Publicado: (2022)

CRISPR/nCas9-Based Genome Editing on GM2 Gangliosidoses Fibroblasts via Non-Viral Vectors
por: Leal, Andrés Felipe, et al.
Publicado: (2022)

The juvenile gangliosidoses: A timeline of clinical change
por: King, Kelly E., et al.
Publicado: (2020)

Functionality of a bicistronic construction containing HEXA and HEXB genes encoding β-hexosaminidase A for cell-mediated therapy of GM2 gangliosidoses
por: Shaimardanova, Alisa A., et al.
Publicado: (2021)

A master protocol to investigate a novel therapy acetyl-l-leucine for three ultra-rare neurodegenerative diseases: Niemann-Pick type C, the GM2 gangliosidoses, and ataxia telangiectasia
por: Fields, T., et al.
Publicado: (2021)

Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study
por: Nestrasil, Igor, et al.
Publicado: (2017)

Optimization of Enzyme Essays to Enhance Reliability of Activity Measurements in Leukocyte Lysates for the Diagnosis of Metachromatic Leukodystrophy and Gangliosidoses
por: Strobel, Sebastian, et al.
Publicado: (2020)

Pinning Down Viral Proteins: A New Prototype for Virus–Host Cell Interaction
por: Kojima, Yoshitsugu, et al.
Publicado: (2010)

Regeneration Process of Ammonia-Absorbed Zirconium Phosphate to Zirconium Phosphate
por: Yamaguchi, Masakuni, et al.
Publicado: (2022)

Omics Profiles of Non-GM Tubers from Transgrafted Potato with a GM Scion
por: Miyahara, Taira, et al.
Publicado: (2023)

Roles of myeloperoxidase and GAPDH in interferon-gamma production of GM-CSF-dependent macrophages
por: Yamaguchi, Rui, et al.
Publicado: (2016)

Distinctive expression patterns of glycoprotein non-metastatic B and folliculin in renal tumors in patients with Birt–Hogg–Dubé syndrome
por: Furuya, Mitsuko, et al.
Publicado: (2015)

Trace Ammonia Equilibrium Pressure of Zirconium Phosphate in Moisture
por: Yamaguchi, Masakuni, et al.
Publicado: (2023)

Salvage Chemotherapy in Patients with Previously Treated Thymic Carcinoma
por: Kaira, Kyoichi, et al.
Publicado: (2021)

Impaired CXCL4 expression in tumor-associated macrophages (TAMs) of ovarian cancers arising in endometriosis
por: Furuya, Mitsuko, et al.
Publicado: (2012)

Clostridium perfringens Alpha-Toxin Induces Gm1a Clustering and Trka Phosphorylation in the Host Cell Membrane
por: Takagishi, Teruhisa, et al.
Publicado: (2015)

Commensal Bacteria Regulate Thymic Aire Expression
por: Nakajima, Akihito, et al.
Publicado: (2014)

Anti-Glycolipid Antibody Examination in Five EAE Models and Theiler’s Virus Model of Multiple Sclerosis: Detection of Anti-GM1, GM3, GM4, and Sulfatide Antibodies in Relapsing-Remitting EAE
por: Moriguchi, Kota, et al.
Publicado: (2023)

Gastric Signet-Ring Cell Carcinoma That Presented as an Elevated Lesion due to Fibromuscular Obliteration in the Lamina Propria
por: Misumi, Yoshitsugu, et al.
Publicado: (2021)

Successful application of angioembolization and thoracic endovascular aortic repair (TEVAR) in a retrograde type-A acute aortic dissection with polytrauma
por: Nagashima, Hideaki, et al.
Publicado: (2021)

GM animals - another GM crops?
por: Bruce, Ann
Publicado: (2007)

Vaccination with Human Induced Pluripotent Stem Cells Creates an Antigen-Specific Immune Response Against HIV-1 gp160
por: Yoshizaki, Shinji, et al.
Publicado: (2011)

Thymic Lymphoma with Thymic Cyst
por: Haque, Anwar Ul, et al.
Publicado: (1994)

Identification of miR-30d as a novel prognostic maker of prostate cancer
por: Kobayashi, Naohito, et al.
Publicado: (2012)

Surgical resection of a rapidly growing pulmonary spindle cell carcinoma by robot-assisted thoracoscopic surgery: a case report
por: Koen, Akihiro, et al.
Publicado: (2021)

Eutectic Phenomenon of LiNH(2)-KH Composite in MH-NH(3) Hydrogen Storage System
por: Goshome, Kiyotaka, et al.
Publicado: (2019)

Clinical Features of Neurodevelopmental Outcomes in Children with Preterm Severe Fetal Growth Restriction: A Retrospective Observational Study
por: Motoki, Takahiro, et al.
Publicado: (2022)

Transcriptional and Histological Analyses of the Thymic Developmental Process in the Fetal Pig
por: Suzuki, Shunichi, et al.
Publicado: (2014)

Nitrogen Dissociation via Reaction with Lithium Alloys
por: Yamaguchi, Shotaro, et al.
Publicado: (2017)

The future of starch bioengineering: GM microorganisms or GM plants?
por: Hebelstrup, Kim H., et al.
Publicado: (2015)

Effects of Educating Local Government Officers and Healthcare and Welfare Professionals in Suicide Prevention
por: Kaniwa, Isao, et al.
Publicado: (2012)

Cannot write session to /tmp/vufind_sessions/sess_q9ocromvimi5rtf3ugv744hh8b