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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...
Autores principales: | Kreiser, Topaz, Sogolovsky-Bard, Ilana, Zaguri, Dor, Shaham-Niv, Shira, Laor Bar-Yosef, Dana, Gazit, Ehud |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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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 |
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