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

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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Bibliographic Details
Main Authors: Topaz Kreiser, Ilana Sogolovsky-Bard, Dor Zaguri, Shira Shaham-Niv, Dana Laor Bar-Yosef, Ehud Gazit
Format: Article
Language:English
Published: MDPI AG 2023-11-01
Series:International Journal of Molecular Sciences
Subjects:
BCAAs
self-assembly
amyloid-like structures
metabolite amyloids
metabolostasis
MSUD pathology
Online Access:https://www.mdpi.com/1422-0067/24/21/15999

Internet

https://www.mdpi.com/1422-0067/24/21/15999

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