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Metabolomics profiling reveals profound metabolic impairments in mice and patients with Sandhoff disease

Sandhoff disease (SD) results from mutations in the HEXB gene, subsequent deficiency of N-acetyl-β-hexosaminidase (Hex) and accumulation of GM2 gangliosides. SD leads to progressive neurodegeneration and early death. However, there is a lack of established SD biomarkers, while the pathogenesis etiol...

Ausführliche Beschreibung

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Bibliographische Detailangaben
Veröffentlicht in:Mol Genet Metab
Hauptverfasser: Ou, Li, Przybilla, Michael J, Whitley, Chester B
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2018
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6365207/
https://ncbi.nlm.nih.gov/pubmed/30236619
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2018.09.005
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