Complement factor: Difference between revisions

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Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky, Jaime Prilusky

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-<StructureSection load='3oxu' size='340' side='right' caption='Structure of human complement factor H 19-20 sushi domains (yellow, magenta, cyan) complex with complement C3 (grey, green, pink) and glycerol (PDB code [[3oxu]]).' scene=''>
+<StructureSection load='3oxu' size='340' side='right' caption='Structure of human complement factor H 19-20 sushi domains (magenta) complex with complement C3 (green) and glycerol (PDB code [[3oxu]]).' scene='59/594535/Cv/1'>
 == Function ==
-'''Complement factor H''' (CFH) regulates the complement system which is part of the immune response.  CFH protects cells by preventing the activation of the complement system when it is not needed.  CFH contains 20 short consensus repeats (sushi domains).
+*'''Complement factor H''' (CFH) regulates the complement system which is part of the immune response.  CFH protects cells by preventing the activation of the complement system when it is not needed.  CFH contains 20 short consensus repeats (sushi domains, i.e., a 5 strand β-sandwich arrangement)<ref>PMID:15163532</ref>.
+*'''Complement factor D''' (CFD) and '''Complement factor B''' (CFB) regulate a key step in the activation of the alternative complement pathway.  CFD is a member of the chymotrypsin family of serine proteases.
+*'''Complement factor I''' (CFI) is a serum protease that inhibits all complement pathways.
+*'''Complement factor P''' (CFP) is a positive regulator of the alternative complement pathway<ref>PMID:28480349</ref>.
 == Disease ==
-CFH mutations are involved in dense deposit disease, age-related macular degeneration, atypical hemolytic-uremic syndrome and the kidney disorder glomerulonephritis.
+CFH mutations are involved in dense deposit disease, age-related macular degeneration, atypical hemolytic-uremic syndrome, schizophrenia, stroke and the kidney disorder glomerulonephritis.  CFD and CFI are implicated in age-related macular degeneration<ref>PMID:22003108</ref>, <ref>PMID:25352734</ref>.
 == Relevance ==
+CFH is recruited by human pathogens resulting in increased virulence.  CFB is a novel target for treatment of cardiometabolic disease<ref>PMID:28739975</ref>.
 == Structural highlights ==
-==3D structures of complement factor H==
+The <scene name='59/594535/Cv/5'>sushi domains are ca. 60 amino acid long containing 2 Cys-Cys bonds</scene>.
+*<scene name='59/594535/Cv/6'>Cys-Cys bonds</scene>.
-Updated on {{REVISIONDAY2}}-{{MONTHNAME|{{REVISIONMONTH}}}}-{{REVISIONYEAR}}
-[[1haq]], [[3gau]], [[3gav]], [[3gaw]] – hCFH – human - NMR<br />
-[[2rlp]] – hCFH 1-2 sushi domains - NMR <br />
-[[2rlq]] – hCFH 2-3 sushi domains - NMR <br />
-[[2qfg]] – hCFH 1-5 sushi domains  <br />
-[[2yby]] – mCFH 6-8 sushi domains (mutant) - mouse<br />
-[[2ic4]] – hCFH 6-8 sushi domains (mutant) <br />
-[[2jgw]] – hCFH 7 sushi domain – NMR<br />
-[[2jgx]] – hCFH 7 sushi domain (mutant) <br />
-[[4b2r]] – hCFH 10-11 sushi domains - NMR <br />
-[[4b2s]] – hCFH 11-12 sushi domains - NMR <br />
-[[2kms]] – hCFH 12-13 sushi domains – NMR<br />
-[[1hfi]] – hCFH 15 sushi domain – NMR<br />
-[[1hfh]] – hCFH 15-16 sushi domains – NMR<br />
-[[2qfh]] – hCFH 16-20 sushi domains - NMR <br />
-[[3sw0]] – hCFH 18-20 sushi domains <br />
-[[2bzm]] – hCFH 19-20 sushi domains – NMR<br />
-[[3kzj]] – hCFH 19-20 sushi domains (mutant) – NMR<br />
-[[2g7i]] – hCFH 19-20 sushi domains <br />
-[[3kxv]], [[3r62]] – hCFH 19-20 sushi domains (mutant) <br />
-===Complement factor H complexes===
-[[2wii]] – hCFH 1-4 sushi domains + complement C3 <br />
-[[2w80]], [[2w81]], [[4ayd]], [[4aye]], [[4ayi]], [[4aym]] – hCFH 6-7 sushi domains + factor H binding protein <br />
-[[2uwn]], [[2v8e]] – hCFH 6-8 sushi domains + sucrose octasulfate <br />
-[[4k12]] – hCFH 9 sushi domain + choline binding protein A <br />
-[[3oxu]] – hCFH 19-20 sushi domains + complement C3 <br />
-[[2xqw]] – hCFH 19-20 sushi domains (mutant) + complement C3 <br />
-[[4j38]] – hCFH 19-20 sushi domains (mutant) + outer surface protein E <br />
-[[4k12]] – hCFH 9 sushi domain + choline binding protein A <br />
+==3D structures of complement factor==
+[[Complement factor 3D structures]]
+</StructureSection>
 == References ==