1mzw
Crystal structure of a U4/U6 snRNP complex between human spliceosomal cyclophilin H and a U4/U6-60K peptide
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OverviewOverview
The spliceosomal cyclophilin H is a specific component of the human U4/U6, small nuclear ribonucleoprotein particle, interacting with homologous, sequences in the proteins U4/U6-60K and hPrp18 during pre-mRNA splicing., We determined the crystal structure of the complex comprising cyclophilin, H and the cognate domain of U4/U6-60K. The 31 amino acid fragment of, U4/U6-60K is bound to a region remote from the cyclophilin active site., Residues Ile118-Phe121 of U4/U6-60K expand the central beta-sheet of, cyclophilin H and the side-chain of Phe121 inserts into a hydrophobic, cavity. Concomitantly, in the crystal the cyclophilin H active site is, occupied by the N terminus of a neighboring cyclophilin H molecule in a, substrate-like manner, indicating the capacity of joint binding to a, substrate and to U4/U6-60K. Free and complexed cyclophilin H have, virtually identical conformations suggesting that the U4/U6-60K binding, site is pre-shaped and the peptidyl-prolyl-cis/trans isomerase activity is, unaffected by complex formation. The complex defines a novel, protein-protein interaction mode for a cyclophilin, allowing cyclophilin H, to mediate interactions between different proteins inside the spliceosome, or to initiate from its binding platforms isomerization or chaperoning, activities.
DiseaseDisease
Known diseases associated with this structure: Cardiomyopathy, dilated OMIM:[605906], Cardiomyopathy, dilated, with left ventricular noncompaction OMIM:[605906], Myopathy, myofibrillar, ZASP-related OMIM:[605906]
About this StructureAbout this Structure
1MZW is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
Crystal structure of a complex between human spliceosomal cyclophilin H and a U4/U6 snRNP-60K peptide., Reidt U, Wahl MC, Fasshauer D, Horowitz DS, Luhrmann R, Ficner R, J Mol Biol. 2003 Aug 1;331(1):45-56. PMID:12875835
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