A novel human model of the neurodegenerative disease GM1 gangliosidosis using induced pluripotent stem cells demonstrates inflammasome activation.

doi:10.1002/path.4551

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Article

A novel human model of the neurodegenerative disease GM1 gangliosidosis using induced pluripotent stem cells demonstrates inflammasome activation.

The Journal of pathology - 1 Sept 2015

Son Mi-Young, Kwak Jae Eun, Seol Binna, Lee Da Yong, Jeon Hyejin, Cho Yee Sook

Abstract excerpt

GM1 gangliosidosis (GM1) is an inherited neurodegenerative disorder caused by mutations in the lysosomal β-galactosidase (β-gal) gene. Insufficient β-gal activity leads to abnormal accumulation of GM1 gangliosides in tissues, particularly in the central nervous system, resulting in progressive neurodegeneration. Here, we report an in vitro human GM1 model, based on induced pluripotent stem cell (iPSC) technology....

Topics

Animals
Biomarkers
Cell Line
Cell Shape
Cellular Reprogramming
Gangliosidosis, GM1
Genotype
Humans
Immunologic Factors
Induced Pluripotent Stem Cells
Inflammasomes

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Interleukin 1 Receptor Antagonist Protein

Lysosomes

Mice, Inbred C57BL

Neural Stem Cells

Phenotype

Signal Transduction

Time Factors

Source

Open in PubMed

Identifiers

PMID: 25925601
DOI: 10.1002/path.4551

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