1kcq

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{{STRUCTURE_1kcq| PDB=1kcq | SCENE= }}
{{STRUCTURE_1kcq| PDB=1kcq | SCENE= }}
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'''Human Gelsolin Domain 2 with a Cd2+ bound'''
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===Human Gelsolin Domain 2 with a Cd2+ bound===
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==Overview==
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Mutations in domain 2 (D2, residues 151-266) of the actin-binding protein gelsolin cause familial amyloidosis-Finnish type (FAF). These mutations, D187N or D187Y, lead to abnormal proteolysis of plasma gelsolin at residues 172-173 and a second hydrolysis at residue 243, resulting in an amyloidogenic fragment. Here we present the structure of human gelsolin D2 at 1.65 A and find that Asp 187 is part of a Cd2+ metal-binding site. Two Ca2+ ions are required for a conformational transition of gelsolin to its active form. Differential scanning calorimetry (DSC) and molecular dynamics (MD) simulations suggest that the Cd2+-binding site in D2 is one of these two Ca2+-binding sites and is essential to the stability of D2. Mutation of Asp 187 to Asn disrupts Ca2+ binding in D2, leading to instabilities upon Ca2+ activation. These instabilities make the domain a target for aberrant proteolysis, thereby enacting the first step in the cascade leading to FAF.
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The line below this paragraph, {{ABSTRACT_PUBMED_11753432}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 11753432 is the PubMed ID number.
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{{ABSTRACT_PUBMED_11753432}}
==About this Structure==
==About this Structure==
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[[Category: Familial amyloidosis--finnish type]]
[[Category: Familial amyloidosis--finnish type]]
[[Category: Metal binding]]
[[Category: Metal binding]]
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Revision as of 07:08, 2 July 2008

Image:1kcq.png

Template:STRUCTURE 1kcq

Human Gelsolin Domain 2 with a Cd2+ bound

Template:ABSTRACT PUBMED 11753432

About this Structure

1KCQ is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Loss of a metal-binding site in gelsolin leads to familial amyloidosis-Finnish type., Kazmirski SL, Isaacson RL, An C, Buckle A, Johnson CM, Daggett V, Fersht AR, Nat Struct Biol. 2002 Feb;9(2):112-6. PMID:11753432

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