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3ior, resolution 3.60Å ()
Ligands: ,
Gene: malE, b4034, JW3994, HTT, HD, IT15 (Escherichia coli K-12)
Related: 3io4, 3io6, 3iot, 3iou, 3iov, 3iow
Resources: FirstGlance, OCA, PDBsum, RCSB
Coordinates: save as pdb, mmCIF, xml


Huntingtin amino-terminal region with 17 Gln residues - crystal C95

Publication Abstract from PubMed

Huntington's disease is a genetic neurodegenerative disorder resulting from polyglutamine (polyQ) expansion (>36Q) within the first exon of Huntingtin (Htt) protein. We applied X-ray crystallography to determine the secondary structure of the first exon (EX1) of Htt17Q. The structure of Htt17Q-EX1 consists of an amino-terminal alpha helix, poly17Q region, and polyproline helix formed by the proline-rich region. The poly17Q region adopts multiple conformations in the structure, including alpha helix, random coil, and extended loop. The conformation of the poly17Q region is influenced by the conformation of neighboring protein regions, demonstrating the importance of the native protein context. We propose that the conformational flexibility of the polyQ region observed in our structure is a common characteristic of many amyloidogenic proteins. We further propose that the pathogenic polyQ expansion in the Htt protein increases the length of the random coil, which promotes aggregation and facilitates abnormal interactions with other proteins in cells.

Secondary structure of Huntingtin amino-terminal region., Kim MW, Chelliah Y, Kim SW, Otwinowski Z, Bezprozvanny I, Structure. 2009 Sep 9;17(9):1205-12. PMID:19748341

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

About this Structure

3ior is a 3 chain structure with sequence from Escherichia coli k-12. Full crystallographic information is available from OCA.

See Also


  • Kim MW, Chelliah Y, Kim SW, Otwinowski Z, Bezprozvanny I. Secondary structure of Huntingtin amino-terminal region. Structure. 2009 Sep 9;17(9):1205-12. PMID:19748341 doi:10.1016/j.str.2009.08.002

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