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PMID:15893665

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Citation

de Chiara, C, Menon, RP, Adinolfi, S, de Boer, J, Ktistaki, E, Kelly, G, Calder, L, Kioussis, D and Pastore, A (2005) The AXH domain adopts alternative folds the solution structure of HBP1 AXH. Structure 13:743-53

Abstract

AXH is a protein module identified in two unrelated families that comprise the transcriptional repressor HBP1 and ataxin-1 (ATX1), the protein responsible for spinocerebellar ataxia type-1 (SCA1). SCA1 is a neurodegenerative disorder associated with protein misfolding and formation of toxic intranuclear aggregates. We have solved the structure in solution of monomeric AXH from HBP1. The domain adopts a nonclassical permutation of an OB fold and binds nucleic acids, a function previously unidentified for this region of HBP1. Comparison of HBP1 AXH with the crystal structure of dimeric ATX1 AXH indicates that, despite the significant sequence homology, the two proteins have different topologies, suggesting that AXH has chameleon properties. We further demonstrate that HBP1 AXH remains monomeric, whereas the ATX1 dimer spontaneously aggregates and forms fibers. Our results describe an entirely novel, to our knowledge, example of a chameleon fold and suggest a link between these properties and the SCA1 pathogenesis.

Links

PubMed Online version:10.1016/j.str.2005.02.016

Keywords

Amino Acid Sequence; Animals; Ataxin-1; Ataxins; Binding Sites; High Mobility Group Proteins/chemistry; High Mobility Group Proteins/metabolism; Humans; Molecular Sequence Data; Nerve Tissue Proteins/chemistry; Nerve Tissue Proteins/metabolism; Nuclear Proteins/chemistry; Nuclear Proteins/metabolism; Protein Folding; Protein Structure, Tertiary; RNA/metabolism; RNA-Binding Proteins/chemistry; RNA-Binding Proteins/metabolism; Repressor Proteins/chemistry; Repressor Proteins/metabolism; Sequence Alignment; Solutions; Spinocerebellar Ataxias/metabolism

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:ATX1

enables

GO:0042802: identical protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P54253

F

Seeded From UniProt

complete

Notes

See also

References

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