2oay: Difference between revisions

Line 1:

~~[[Image:2oay.png|left|200px]]~~

===Crystal structure of latent human C1-inhibitor===

===~~Crystal structure~~ of ~~latent human~~ C1-~~inhibitor===~~

==Disease==

[[http://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN]] Defects in SERPING1 are the cause of hereditary angioedema (HAE) [MIM:[http://omim.org/entry/106100 106100]]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional.<ref>PMID:12773530</ref><ref>PMID:7814636</ref><ref>PMID:3178731</ref><ref>PMID:2365061</ref><ref>PMID:2296585</ref><ref>PMID:2118657</ref>[:]<ref>PMID:1451784</ref><ref>PMID:1363816</ref><ref>PMID:8172583</ref><ref>PMID:7883978</ref><ref>PMID:8529136</ref><ref>PMID:8755917</ref><ref>PMID:14635117</ref><ref>PMID:16409206</ref><ref>PMID:22994404</ref>

~~{{ABSTRACT_PUBMED_17488724}}~~

==Function==

[[http://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN]] Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.<ref>PMID:8495195</ref>

==About this Structure==

Line 11:

Line 13:

==Reference==

[[Category: Homo sapiens]]

[[Category: Beinrohr, L.]]

Navigation menu

Page actions

Page actions

Personal tools

Navigation

Search

Tools

@@ Line 1: / Line 1: @@
-[[Image:2oay.png|left|200px]]
 {{STRUCTURE_2oay|  PDB=2oay  |  SCENE=  }}
+===Crystal structure of latent human C1-inhibitor===
+{{ABSTRACT_PUBMED_17488724}}
-===Crystal structure of latent human C1-inhibitor===
+==Disease==
+[[http://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN]] Defects in SERPING1 are the cause of hereditary angioedema (HAE) [MIM:[http://omim.org/entry/106100 106100]]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional.<ref>PMID:12773530</ref><ref>PMID:7814636</ref><ref>PMID:3178731</ref><ref>PMID:2365061</ref><ref>PMID:2296585</ref><ref>PMID:2118657</ref>[:]<ref>PMID:1451784</ref><ref>PMID:1363816</ref><ref>PMID:8172583</ref><ref>PMID:7883978</ref><ref>PMID:8529136</ref><ref>PMID:8755917</ref><ref>PMID:14635117</ref><ref>PMID:16409206</ref><ref>PMID:22994404</ref>
-{{ABSTRACT_PUBMED_17488724}}
+==Function==
+[[http://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN]] Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.<ref>PMID:8495195</ref>
 ==About this Structure==
@@ Line 11: / Line 13: @@
 ==Reference==
-<ref group="xtra">PMID:017488724</ref><references group="xtra"/>
+<ref group="xtra">PMID:017488724</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Beinrohr, L.]]