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Structure, Function, Folding, and Aggregation of a Neuroferritinopathy-related Ferritin Variant.

08:00 EDT 15th April 2019 | BioPortfolio

Summary of "Structure, Function, Folding, and Aggregation of a Neuroferritinopathy-related Ferritin Variant."

Neuroferritinopathy is a rare, adult-onset, dominantly inherited movement disorder caused by mutations in ferritin gene. A ferritin light chain variant related to neuroferritinopathy, in which alanine 96 is replaced with threonine (A96T), was expressed in Escherichia coli, purified, and characterized. The circular dichroism spectrum, analytical ultracentrifuge, and small-angle X-ray scattering studies have shown that both subunit structure and assembly of A96T are the same as those of wild-type human ferritin light chain (HuFTL). Iron-incorporation ability was also comparable to that of HuFTL. Although the structural stability against heat, acid, and denaturant was reduced, the structure was sufficiently stable under physiological conditions. The most remarkable defect observed for A96T was lower refolding efficiency and higher propensity to aggregate. The possible relationship between folding deficiency and disease is discussed.

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Name: Biochemistry
ISSN: 1520-4995
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