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Branched-Chain Amino Acid Assembly into Amyloid-like Fibrils Provides a New Paradigm for Maple Syrup Urine Disease Pathology

Inborn error of metabolism disorders (IEMs) are a family of diseases resulting from single-gene mutations that lead to the accumulation of metabolites that are usually toxic or interfere with normal cell function. The etiological link between metabolic alteration and the symptoms of IEMs is still el...

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Detalles Bibliográficos
Autores principales: Kreiser, Topaz, Sogolovsky-Bard, Ilana, Zaguri, Dor, Shaham-Niv, Shira, Laor Bar-Yosef, Dana, Gazit, Ehud
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10650742/
https://www.ncbi.nlm.nih.gov/pubmed/37958982
http://dx.doi.org/10.3390/ijms242115999