EGFR activation triggers cellular hypertrophy and lysosomal disease in NAGLU-depleted cardiomyoblasts, mimicking the hallmarks of mucopolysaccharidosis IIIB

Mucopolysaccharidosis (MPS) IIIB is an inherited lysosomal storage disease caused by the deficiency of the enzyme α-N-acetylglucosaminidase (NAGLU) required for heparan sulfate (HS) degradation. The defective lysosomal clearance of undigested HS results in dysfunction of multiple tissues and organs....

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Vydáno v:Cell Death Dis
Hlavní autoři: De Pasquale, Valeria, Pezone, Antonio, Sarogni, Patrizia, Tramontano, Alfonso, Schiattarella, Gabriele Giacomo, Avvedimento, Vittorio Enrico, Paladino, Simona, Pavone, Luigi Michele
Médium: Artigo
Jazyk:Inglês
Vydáno: Nature Publishing Group UK 2018
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Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5833457/
https://ncbi.nlm.nih.gov/pubmed/29348482
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41419-017-0187-0
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