Abstract:
:Phenylketonuria (PKU) is characterized by phenylalanine accumulation and progressive mental retardation caused by an unknown mechanism. We demonstrate that at pathological concentrations, phenylalanine self-assembles into fibrils with amyloid-like morphology and well-ordered electron diffraction. These assemblies are specifically recognized by antibodies, show cytotoxicity that can be neutralized by the antibodies and are present in the hippocampus of model mice and in parietal cortex brain tissue from individuals with PKU. This is, to our knowledge, the first demonstration that a single amino acid can form amyloid-like deposits, suggesting a new amyloidosis-like etiology for PKU.
journal_name
Nat Chem Bioljournal_title
Nature chemical biologyauthors
Adler-Abramovich L,Vaks L,Carny O,Trudler D,Magno A,Caflisch A,Frenkel D,Gazit Edoi
10.1038/nchembio.1002subject
Has Abstractpub_date
2012-08-01 00:00:00pages
701-6issue
8eissn
1552-4450issn
1552-4469pii
nchembio.1002journal_volume
8pub_type
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