4.4 Article

Trehalose consumption ameliorates pathogenesis in an inducible mouse model of the Fragile X-associated tremor/ataxia syndrome

相关参考文献

注意:仅列出部分参考文献,下载原文获取全部文献信息。
Article Neurosciences

Prevalence of Fragile X-Associated Tremor/Ataxia Syndrome in Patients with Cerebellar Ataxia in Japan

Yujiro Higuchi et al.

Summary: This study aimed to determine the prevalence of FXTAS in Japanese patients with cerebellar ataxia and describe their clinical characteristics. Among 995 patients screened, 3 were identified as having FMR1 premutation and meeting the diagnostic criteria for FXTAS.

CEREBELLUM (2022)

Article Multidisciplinary Sciences

Short antisense oligonucleotides alleviate the pleiotropic toxicity of RNA harboring expanded CGG repeats

Magdalena Derbis et al.

Summary: The study demonstrates that short antisense oligonucleotide steric blockers targeting rCGG(exp) in FXTAS cells can reduce R-loop formation and correct miRNA biogenesis, leading to improved motor behavior and gene expression profile in animal models with minimal toxicity. This suggests a potential therapeutic approach for the treatment of Fragile X-associated tremor/ataxia syndrome.

NATURE COMMUNICATIONS (2021)

Article Immunology

Trehalose-Induced Remodelling of the Human Microbiota Affects Clostridioides difficile Infection Outcome in an In Vitro Colonic Model: A Pilot Study

Anthony M. Buckley et al.

Summary: The study demonstrates that trehalose supplementation remodels the human gut microbiota, preventing simulated C. difficile infection. The microbial functions in the trehalose model differ from those in other models, potentially leading to the prevention of CDI.

FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY (2021)

Letter Clinical Neurology

Oral trehalose maybe helpful for patients with spinocerebellar ataxia 3 and should be better evaluated

M. A. Noorasyikin et al.

PARKINSONISM & RELATED DISORDERS (2020)

Editorial Material Pharmacology & Pharmacy

Drug Screen Tugs at Common Thread for Repeat Disorders

Kaalak Reddy et al.

TRENDS IN PHARMACOLOGICAL SCIENCES (2020)

Article Medicine, Research & Experimental

Trehalose alleviates the phenotype of Machado-Joseph disease mouse models

Magda M. Santana et al.

JOURNAL OF TRANSLATIONAL MEDICINE (2020)

Article Biochemistry & Molecular Biology

FMRpoIyG alters mitochondria' transcripts level and respiratory chain complex assembly in Fragile X associated tremor/ataxia syndrome [FXTAS]

Dhruv Gohel et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2019)

Article Pharmacology & Pharmacy

Beneficial Effects of Trehalose on Striatal Dopaminergic Deficits in Rodent and Primate Models of Synucleinopathy in Parkinson's Disease

Patrick A. Howson et al.

JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS (2019)

Article Neurosciences

Composition of the Intranuclear Inclusions of Fragile X-associated Tremor/Ataxia Syndrome

Lisa Ma et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2019)

Article Food Science & Technology

Trehalose as a cryoprotectant in freeze-dried wheat sourdough production

Raquel Facco Stefanello et al.

LWT-FOOD SCIENCE AND TECHNOLOGY (2018)

Article Biochemistry & Molecular Biology

Trehalose protects against oxidative stress by regulating the Keap1-Nrf2 and autophagy pathways

Yuhei Mizunoe et al.

REDOX BIOLOGY (2018)

Article Food Science & Technology

Honey, trehalose and erythritol as sucrose-alternative sweeteners for artisanal ice cream. A pilot study

Maria Eletta Moriano et al.

LWT-FOOD SCIENCE AND TECHNOLOGY (2017)

Article Multidisciplinary Sciences

Exploiting macrophage autophagy-lysosomal biogenesis as a therapy for atherosclerosis

Ismail Sergin et al.

NATURE COMMUNICATIONS (2017)

Review Clinical Neurology

Fragile X-associated tremor/ataxia syndrome - features, mechanisms and management

Randi J. Hagerman et al.

NATURE REVIEWS NEUROLOGY (2016)

Editorial Material Biochemistry & Molecular Biology

Mystery solved: Trehalose kickstarts autophagy by blocking glucose transport

Pablo Mardones et al.

SCIENCE SIGNALING (2016)

Editorial Material Biochemistry & Molecular Biology

Mystery solved: Trehalose kickstarts autophagy by blocking glucose transport

Pablo Mardones et al.

SCIENCE SIGNALING (2016)

Article Biochemistry & Molecular Biology

Trehalose intake induces chaperone molecules along with autophagy in a mouse model of Lewy body disease

Kunikazu Tanji et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2015)

Meeting Abstract Pharmacology & Pharmacy

CHARACTERIZATION OF BLOOD GLUCOSE & GLYCOSURIA FOLLOWING IV TREHALOSE (CABALETTA) ADMINISTRATION FOR OPMD

H. Vornovitzki et al.

CLINICAL THERAPEUTICS (2015)

Article Biochemistry & Molecular Biology

Reversibility of neuropathology and motor deficits in an inducible mouse model for FXTAS

Renate K. Hukema et al.

HUMAN MOLECULAR GENETICS (2015)

Article Biochemistry & Molecular Biology

Trehalose Glycopolymers as Excipients for Protein Stabilization

Juneyoung Lee et al.

BIOMACROMOLECULES (2013)

Article Biochemistry & Molecular Biology

Fibroblast phenotype in male carriers of FMR1 premutation alleles

Dolores Garcia-Arocena et al.

HUMAN MOLECULAR GENETICS (2010)

Article Biochemistry & Molecular Biology

Penetrance of FMR1 premutation associated pathologies in fragile X syndrome families

Laia Rodriguez-Revenga et al.

EUROPEAN JOURNAL OF HUMAN GENETICS (2009)

Article Biochemistry & Molecular Biology

p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

Serhiy Pankiv et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2007)

Article Biochemistry & Molecular Biology

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

M Tanaka et al.

NATURE MEDICINE (2004)

Article Medicine, General & Internal

Penetrance of the fragile X - Associated tremor/ataxia syndrome in a premutation carrier population

S Jacquemont et al.

JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2004)

Review Food Science & Technology

Trehalose: a review of properties, history of use and human tolerance, and results of multiple safety studies

AB Richards et al.

FOOD AND CHEMICAL TOXICOLOGY (2002)