4f27: Difference between revisions
No edit summary |
No edit summary |
||
| Line 1: | Line 1: | ||
{{STRUCTURE_4f27| PDB=4f27 | SCENE= }} | {{STRUCTURE_4f27| PDB=4f27 | SCENE= }} | ||
===Crystal structures reveal the multi-ligand binding mechanism of the Staphylococcus aureus ClfB=== | |||
{{ABSTRACT_PUBMED_22719251}} | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/FIBA_HUMAN FIBA_HUMAN]] Defects in FGA are a cause of congenital afibrinogenemia (CAFBN) [MIM:[http://omim.org/entry/202400 202400]]. This is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Note=The majority of cases of afibrinogenemia are due to truncating mutations. Variations in position Arg-35 (the site of cleavage of fibrinopeptide a by thrombin) leads to alpha-dysfibrinogenemias. Defects in FGA are a cause of amyloidosis type 8 (AMYL8) [MIM:[http://omim.org/entry/105200 105200]]; also known as systemic non-neuropathic amyloidosis or Ostertag-type amyloidosis. AMYL8 is a hereditary generalized amyloidosis due to deposition of apolipoprotein A1, fibrinogen and lysozyme amyloids. Viscera are particularly affected. There is no involvement of the nervous system. Clinical features include renal amyloidosis resulting in nephrotic syndrome, arterial hypertension, hepatosplenomegaly, cholestasis, petechial skin rash.<ref>PMID:8097946</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/FIBA_HUMAN FIBA_HUMAN]] Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation. | |||
==About this Structure== | ==About this Structure== | ||
[[4f27]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Staphylococcus_aureus Staphylococcus aureus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4F27 OCA]. | [[4f27]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Staphylococcus_aureus Staphylococcus aureus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4F27 OCA]. | ||
==See Also== | |||
*[[Fibrinogen binding protein|Fibrinogen binding protein]] | |||
==Reference== | ==Reference== | ||
<ref group="xtra">PMID:022719251</ref><references group="xtra"/> | <ref group="xtra">PMID:022719251</ref><references group="xtra"/><references/> | ||
[[Category: Staphylococcus aureus]] | [[Category: Staphylococcus aureus]] | ||
[[Category: Chen, Y G.]] | [[Category: Chen, Y G.]] | ||
Revision as of 01:00, 25 March 2013
Crystal structures reveal the multi-ligand binding mechanism of the Staphylococcus aureus ClfBCrystal structures reveal the multi-ligand binding mechanism of the Staphylococcus aureus ClfB
Template:ABSTRACT PUBMED 22719251
DiseaseDisease
[FIBA_HUMAN] Defects in FGA are a cause of congenital afibrinogenemia (CAFBN) [MIM:202400]. This is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Note=The majority of cases of afibrinogenemia are due to truncating mutations. Variations in position Arg-35 (the site of cleavage of fibrinopeptide a by thrombin) leads to alpha-dysfibrinogenemias. Defects in FGA are a cause of amyloidosis type 8 (AMYL8) [MIM:105200]; also known as systemic non-neuropathic amyloidosis or Ostertag-type amyloidosis. AMYL8 is a hereditary generalized amyloidosis due to deposition of apolipoprotein A1, fibrinogen and lysozyme amyloids. Viscera are particularly affected. There is no involvement of the nervous system. Clinical features include renal amyloidosis resulting in nephrotic syndrome, arterial hypertension, hepatosplenomegaly, cholestasis, petechial skin rash.[1]
FunctionFunction
[FIBA_HUMAN] Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation.
About this StructureAbout this Structure
4f27 is a 2 chain structure with sequence from Staphylococcus aureus. Full crystallographic information is available from OCA.
See AlsoSee Also
ReferenceReference
- ↑ Xiang H, Feng Y, Wang J, Liu B, Chen Y, Liu L, Deng X, Yang M. Crystal Structures Reveal the Multi-Ligand Binding Mechanism of Staphylococcus aureus ClfB. PLoS Pathog. 2012 Jun;8(6):e1002751. Epub 2012 Jun 14. PMID:22719251 doi:10.1371/journal.ppat.1002751
- ↑ Benson MD, Liepnieks J, Uemichi T, Wheeler G, Correa R. Hereditary renal amyloidosis associated with a mutant fibrinogen alpha-chain. Nat Genet. 1993 Mar;3(3):252-5. PMID:8097946 doi:http://dx.doi.org/10.1038/ng0393-252