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PDBsum entry 5acm
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Immune system
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PDB id
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5acm
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References listed in PDB file
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Key reference
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Title
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Inhibition by small-Molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain.
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Authors
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B.Brumshtein,
S.R.Esswein,
L.Salwinski,
M.L.Phillips,
A.T.Ly,
D.Cascio,
M.R.Sawaya,
D.S.Eisenberg.
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Ref.
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Elife, 2015,
4,
e10935.
[DOI no: ]
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PubMed id
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Abstract
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Overproduction of immunoglobulin light chains leads to systemic amyloidosis, a
lethal disease characterized by the formation of amyloid fibrils in patients'
tissues. Excess light chains are in equilibrium between dimers and less stable
monomers which can undergo irreversible aggregation to the amyloid state. The
dimers therefore must disassociate into monomers prior to forming amyloid
fibrils. Here we identify ligands that inhibit amyloid formation by stabilizing
the Mcg light chain variable domain dimer and shifting the equilibrium away from
the amyloid-prone monomer.
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