Amyloid Mimicking Assemblies of Homogentisic Acid, N-Acetyl Aspartic Acid and Isovaleric Acid and its Implications in associated Rare Metabolic Diseases
Abstract:Herein, we have studied the self-assembling and aggregation properties of Homogentisic acid (HA), N-acetyl aspartic Acid (NAA) and Isovaleric acid (IVA) with the aim to understand the effect of aggregation of these metabolites on the toxicities associated with rare metabolic diseases caused by their accumulation. To our surprise, we noted aggregation of HA, NAA and IVA follow amyloidogenic pathway and as these metabolites are aged small globules tend to merge and form fibrillar aggregates. These assemblies wer… Show more
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