1cyn
From Proteopedia
(Difference between revisions)
| Line 1: | Line 1: | ||
| - | [[Image:1cyn.png|left|200px]] | ||
| - | |||
{{STRUCTURE_1cyn| PDB=1cyn | SCENE= }} | {{STRUCTURE_1cyn| PDB=1cyn | SCENE= }} | ||
| - | |||
===CYCLOPHILIN B COMPLEXED WITH [D-(CHOLINYLESTER)SER8]-CYCLOSPORIN=== | ===CYCLOPHILIN B COMPLEXED WITH [D-(CHOLINYLESTER)SER8]-CYCLOSPORIN=== | ||
| + | {{ABSTRACT_PUBMED_8197205}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/PPIB_HUMAN PPIB_HUMAN]] Defects in PPIB are the cause of osteogenesis imperfecta type 9 (OI9) [MIM:[http://omim.org/entry/259440 259440]]. OI9 is a connective tissue disorder characterized by bone fragility, low bone mass and bowing of limbs due to multiple fractures. Short limb dwarfism and blue sclerae are observed in some but not all patients.<ref>PMID:19781681</ref><ref>PMID:20089953</ref> | ||
| + | |||
| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/PPIB_HUMAN PPIB_HUMAN]] PPIases accelerate the folding of proteins. It catalyzes the cis-trans isomerization of proline imidic peptide bonds in oligopeptides. | ||
==About this Structure== | ==About this Structure== | ||
| Line 14: | Line 16: | ||
==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:008197205</ref><ref group="xtra">PMID:008263916</ref><ref group="xtra">PMID:011206060</ref><references group="xtra"/> | + | <ref group="xtra">PMID:008197205</ref><ref group="xtra">PMID:008263916</ref><ref group="xtra">PMID:011206060</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Peptidylprolyl isomerase]] | [[Category: Peptidylprolyl isomerase]] | ||
Revision as of 04:04, 25 March 2013
Contents |
CYCLOPHILIN B COMPLEXED WITH [D-(CHOLINYLESTER)SER8]-CYCLOSPORIN
Template:ABSTRACT PUBMED 8197205
Disease
[PPIB_HUMAN] Defects in PPIB are the cause of osteogenesis imperfecta type 9 (OI9) [MIM:259440]. OI9 is a connective tissue disorder characterized by bone fragility, low bone mass and bowing of limbs due to multiple fractures. Short limb dwarfism and blue sclerae are observed in some but not all patients.[1][2]
Function
[PPIB_HUMAN] PPIases accelerate the folding of proteins. It catalyzes the cis-trans isomerization of proline imidic peptide bonds in oligopeptides.
About this Structure
1cyn is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See Also
Reference
- Mikol V, Kallen J, Walkinshaw MD. X-ray structure of a cyclophilin B/cyclosporin complex: comparison with cyclophilin A and delineation of its calcineurin-binding domain. Proc Natl Acad Sci U S A. 1994 May 24;91(11):5183-6. PMID:8197205
- Mikol V, Kallen J, Pflugl G, Walkinshaw MD. X-ray structure of a monomeric cyclophilin A-cyclosporin A crystal complex at 2.1 A resolution. J Mol Biol. 1993 Dec 20;234(4):1119-30. PMID:8263916 doi:http://dx.doi.org/10.1006/jmbi.1993.1664
- Carpentier M, Allain F, Haendler B, Slomianny MC, Spik G. Delineation of the calcineurin-interacting region of cyclophilin B. Protein Sci. 2000 Dec;9(12):2386-93. PMID:11206060 doi:10.1110/ps.9.12.2386
- ↑ van Dijk FS, Nesbitt IM, Zwikstra EH, Nikkels PG, Piersma SR, Fratantoni SA, Jimenez CR, Huizer M, Morsman AC, Cobben JM, van Roij MH, Elting MW, Verbeke JI, Wijnaendts LC, Shaw NJ, Hogler W, McKeown C, Sistermans EA, Dalton A, Meijers-Heijboer H, Pals G. PPIB mutations cause severe osteogenesis imperfecta. Am J Hum Genet. 2009 Oct;85(4):521-7. doi: 10.1016/j.ajhg.2009.09.001. Epub 2009 , Sep 24. PMID:19781681 doi:10.1016/j.ajhg.2009.09.001
- ↑ Barnes AM, Carter EM, Cabral WA, Weis M, Chang W, Makareeva E, Leikin S, Rotimi CN, Eyre DR, Raggio CL, Marini JC. Lack of cyclophilin B in osteogenesis imperfecta with normal collagen folding. N Engl J Med. 2010 Feb 11;362(6):521-8. doi: 10.1056/NEJMoa0907705. Epub 2010 Jan, 20. PMID:20089953 doi:10.1056/NEJMoa0907705
