7pck

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{{Seed}}
 
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[[Image:7pck.png|left|200px]]
 
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{{STRUCTURE_7pck| PDB=7pck | SCENE= }}
{{STRUCTURE_7pck| PDB=7pck | SCENE= }}
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===CRYSTAL STRUCTURE OF WILD TYPE HUMAN PROCATHEPSIN K===
===CRYSTAL STRUCTURE OF WILD TYPE HUMAN PROCATHEPSIN K===
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{{ABSTRACT_PUBMED_10048321}}
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==Disease==
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[[http://www.uniprot.org/uniprot/CATK_HUMAN CATK_HUMAN]] Defects in CTSK are the cause of pycnodysostosis (PKND) [MIM:[http://omim.org/entry/265800 265800]]. PKND is an autosomal recessive osteochondrodysplasia characterized by osteosclerosis and short stature.<ref>PMID:8703060</ref><ref>PMID:9529353</ref><ref>PMID:10491211</ref><ref>PMID:10878663</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/CATK_HUMAN CATK_HUMAN]] Closely involved in osteoclastic bone resorption and may participate partially in the disorder of bone remodeling. Displays potent endoprotease activity against fibrinogen at acid pH. May play an important role in extracellular matrix degradation.
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(as it appears on PubMed at http://www.pubmed.gov), where 10048321 is the PubMed ID number.
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{{ABSTRACT_PUBMED_10048321}}
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==About this Structure==
==About this Structure==
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7PCK is a 4 chains structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7PCK OCA].
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[[7pck]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7PCK OCA].
==Reference==
==Reference==
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<ref group="xtra">PMID:10048321</ref><references group="xtra"/>
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<ref group="xtra">PMID:010048321</ref><references group="xtra"/><references/>
[[Category: Cathepsin K]]
[[Category: Cathepsin K]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Sivaraman, J.]]
[[Category: Sivaraman, J.]]
[[Category: Cysteine protease]]
[[Category: Cysteine protease]]
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[[Category: Hydrolase]]
[[Category: Procathepsin k]]
[[Category: Procathepsin k]]
[[Category: Proregion]]
[[Category: Proregion]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 14:29:31 2009''
 

Revision as of 22:22, 24 March 2013

Template:STRUCTURE 7pck

Contents

CRYSTAL STRUCTURE OF WILD TYPE HUMAN PROCATHEPSIN K

Template:ABSTRACT PUBMED 10048321

Disease

[CATK_HUMAN] Defects in CTSK are the cause of pycnodysostosis (PKND) [MIM:265800]. PKND is an autosomal recessive osteochondrodysplasia characterized by osteosclerosis and short stature.[1][2][3][4]

Function

[CATK_HUMAN] Closely involved in osteoclastic bone resorption and may participate partially in the disorder of bone remodeling. Displays potent endoprotease activity against fibrinogen at acid pH. May play an important role in extracellular matrix degradation.

About this Structure

7pck is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Sivaraman J, Lalumiere M, Menard R, Cygler M. Crystal structure of wild-type human procathepsin K. Protein Sci. 1999 Feb;8(2):283-90. PMID:10048321
  1. ↑ Gelb BD, Shi GP, Chapman HA, Desnick RJ. Pycnodysostosis, a lysosomal disease caused by cathepsin K deficiency. Science. 1996 Aug 30;273(5279):1236-8. PMID:8703060
  2. ↑ Gelb BD, Willner JP, Dunn TM, Kardon NB, Verloes A, Poncin J, Desnick RJ. Paternal uniparental disomy for chromosome 1 revealed by molecular analysis of a patient with pycnodysostosis. Am J Hum Genet. 1998 Apr;62(4):848-54. PMID:9529353 doi:S0002-9297(07)60977-X
  3. ↑ Ho N, Punturieri A, Wilkin D, Szabo J, Johnson M, Whaley J, Davis J, Clark A, Weiss S, Francomano C. Mutations of CTSK result in pycnodysostosis via a reduction in cathepsin K protein. J Bone Miner Res. 1999 Oct;14(10):1649-53. PMID:10491211
  4. ↑ Haagerup A, Hertz JM, Christensen MF, Binderup H, Kruse TA. Cathepsin K gene mutations and 1q21 haplotypes in at patients with pycnodysostosis in an outbred population. Eur J Hum Genet. 2000 Jun;8(6):431-6. PMID:10878663 doi:10.1038/sj.ejhg.5200481

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