8acf

Structure of the argX-117 in complex with a complement C2 fragment at low pHStructure of the argX-117 in complex with a complement C2 fragment at low pH

Structural highlights

8acf is a 4 chain structure with sequence from Homo sapiens and Lama glama. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:	X-ray diffraction, Resolution 1.8Å
Ligands:	, , , , ,
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

CO2_HUMAN Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000. A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections.^[1] ^[2]

Function

CO2_HUMAN Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor 4b to generate the C3 or C5 convertase.

References

↑ Wetsel RA, Kulics J, Lokki ML, Kiepiela P, Akama H, Johnson CA, Densen P, Colten HR. Type II human complement C2 deficiency. Allele-specific amino acid substitutions (Ser189 --> Phe; Gly444 --> Arg) cause impaired C2 secretion. J Biol Chem. 1996 Mar 8;271(10):5824-31. PMID:8621452
↑ Zhu ZB, Atkinson TP, Volanakis JE. A novel type II complement C2 deficiency allele in an African-American family. J Immunol. 1998 Jul 15;161(2):578-84. PMID:9670930

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OCA

[1] Wetsel RA, Kulics J, Lokki ML, Kiepiela P, Akama H, Johnson CA, Densen P, Colten HR. Type II human complement C2 deficiency. Allele-specific amino acid substitutions (Ser189 --> Phe; Gly444 --> Arg) cause impaired C2 secretion. J Biol Chem. 1996 Mar 8;271(10):5824-31. PMID:8621452

[2] Zhu ZB, Atkinson TP, Volanakis JE. A novel type II complement C2 deficiency allele in an African-American family. J Immunol. 1998 Jul 15;161(2):578-84. PMID:9670930

[1]

[2]

8acf

Structure of the argX-117 in complex with a complement C2 fragment at low pHStructure of the argX-117 in complex with a complement C2 fragment at low pH

Structural highlights

Disease

Function

References

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8acf

Structure of the argX-117 in complex with a complement C2 fragment at low pHStructure of the argX-117 in complex with a complement C2 fragment at low pH

Structural highlights

Disease

Function

References

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