3ich

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[[Image:3ich.png|left|200px]]
 
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{{STRUCTURE_3ich| PDB=3ich | SCENE= }}
{{STRUCTURE_3ich| PDB=3ich | SCENE= }}
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===Crystal structure of cyclophilin B at 1.2 A resolution===
===Crystal structure of cyclophilin B at 1.2 A resolution===
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{{ABSTRACT_PUBMED_20801878}}
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{{ABSTRACT_PUBMED_20801878}}
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==Disease==
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[[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>
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==Function==
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[[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==
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==Reference==
==Reference==
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<ref group="xtra">PMID:020801878</ref><references group="xtra"/>
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<ref group="xtra">PMID:020801878</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Peptidylprolyl isomerase]]
[[Category: Peptidylprolyl isomerase]]

Revision as of 20:39, 24 March 2013

Template:STRUCTURE 3ich

Contents

Crystal structure of cyclophilin B at 1.2 A resolution

Template:ABSTRACT PUBMED 20801878

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

3ich is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

Reference

  • Kozlov G, Bastos-Aristizabal S, Maattanen P, Rosenauer A, Zheng F, Killikelly A, Trempe JF, Thomas DY, Gehring K. Structural basis of cyclophilin B binding by the calnexin/calreticulin P-domain. J Biol Chem. 2010 Sep 3. PMID:20801878 doi:10.1074/jbc.M110.160101
  1. ↑ 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
  2. ↑ 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

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