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2rk6

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{{STRUCTURE_2rk6| PDB=2rk6 | SCENE= }}
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'''Structure of E163K DJ-1'''
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===Structure of E163K DJ-1===
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==Overview==
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A number of missense mutations in the oxidative stress response protein DJ-1 are implicated in rare forms of familial Parkinsonism. The best-characterized Parkinsonian DJ-1 missense mutation, L166P, disrupts homodimerization and results in a poorly folded protein. The molecular basis by which the other Parkinsonism-associated mutations disrupt the function of DJ-1, however, is incompletely understood. In this study we show that three different Parkinsonism-associated DJ-1 missense mutations (A104T, E163K, and M26I) reduce the thermal stability of DJ-1 in solution by subtly perturbing the structure of DJ-1 without causing major folding defects or loss of dimerization. Atomic resolution X-ray crystallography shows that the A104T substitution introduces water and a discretely disordered residue into the core of the protein, E163K disrupts a key salt bridge with R145, and M26I causes packing defects in the core of the dimer. The deleterious effect of each Parkinsonism-associated mutation on DJ-1 is dissected by analysis of engineered substitutions (M26L, A104V, and E163K/R145E) that partially alleviate each of the defects introduced by the A104T, E163K and M26I mutations. In total, our results suggest that the protective function of DJ-1 can be compromised by diverse perturbations in its structural integrity, particularly near the junctions of secondary structural elements.
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{{ABSTRACT_PUBMED_18181649}}
==Disease==
==Disease==
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Revision as of 02:16, 28 July 2008

Image:2rk6.png

Template:STRUCTURE 2rk6

Contents

Structure of E163K DJ-1

Template:ABSTRACT PUBMED 18181649

Disease

Known disease associated with this structure: Amyotrophic lateral sclerosis-Parkinsonism/dementia complex 2 OMIM:[602533], Parkinson disease 7, autosomal recessive early-onset OMIM:[602533]

About this Structure

2RK6 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structural Impact of Three Parkinsonism-Associated Missense Mutations on Human DJ-1(,)., Lakshminarasimhan M, Maldonado MT, Zhou W, Fink AL, Wilson MA, Biochemistry. 2008 Feb 5;47(5):1381-92. Epub 2008 Jan 9. PMID:18181649

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