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Brief Report: Deficiency of Complement 1r Subcomponent in Early-Onset Systemic Lupus Erythematosus: The Role of Disease-Modifying Alleles in a Monogenic Disease.

Demirkaya E, Zhou Q, Smith CK, Ombrello MJ, Deuitch N, Tsai WL, Hoffmann P, Remmers EF, Takeuchi M, Park YH, Chae J, Barut K, Simsek D, Adrovic A, Sahin S, Caliskan S, Chandrasekharappa SC, Hasni SA, Ombrello AK, Gadina M, Kastner DL, Kaplan MJ, Kasapcopur O, Aksentijevich I.

Arthritis Rheumatol. 2017 Sep;69(9):1832-1839. doi: 10.1002/art.40158. Epub 2017 Jul 10.


Molecular determinants of the substrate specificity of the complement-initiating protease, C1r.

Wijeyewickrema LC, Yongqing T, Tran TP, Thompson PE, Viljoen JE, Coetzer TH, Duncan RC, Kass I, Buckle AM, Pike RN.

J Biol Chem. 2013 May 31;288(22):15571-80. doi: 10.1074/jbc.M113.451757. Epub 2013 Apr 15.


Interaction between separated consecutive complement control modules of human C1r: implications for dimerization of the full-length protease.

Láng A, Major B, Szilágyi K, Gáspári Z, Gál P, Závodszky P, Perczel A.

FEBS Lett. 2010 Nov 19;584(22):4565-9. doi: 10.1016/j.febslet.2010.10.033. Epub 2010 Oct 21.


Intermodule cooperativity in the structure and dynamics of consecutive complement control modules in human C1r: structural biology.

Láng A, Szilágyi K, Major B, Gál P, Závodszky P, Perczel A.

FEBS J. 2010 Oct;277(19):3986-98. doi: 10.1111/j.1742-4658.2010.07790.x. Epub 2010 Aug 26.


The human complement component C1R gene: the exon-intron structure and the molecular basis of allelic diversity.

Nakagawa M, Yuasa I, Irizawa Y, Umetsu K.

Ann Hum Genet. 2003 May;67(Pt 3):207-15.


Monomeric structures of the zymogen and active catalytic domain of complement protease c1r: further insights into the c1 activation mechanism.

Budayova-Spano M, Grabarse W, Thielens NM, Hillen H, Lacroix M, Schmidt M, Fontecilla-Camps JC, Arlaud GJ, Gaboriaud C.

Structure. 2002 Nov;10(11):1509-19.


Assembly and enzymatic properties of the catalytic domain of human complement protease C1r.

Lacroix M, Ebel C, Kardos J, Dobó J, Gál P, Závodszky P, Arlaud GJ, Thielens NM.

J Biol Chem. 2001 Sep 28;276(39):36233-40. Epub 2001 Jul 9.


Transcriptional analysis of multiple sclerosis brain lesions reveals a complex pattern of cytokine expression.

Baranzini SE, Elfstrom C, Chang SY, Butunoi C, Murray R, Higuchi R, Oksenberg JR.

J Immunol. 2000 Dec 1;165(11):6576-82.


Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling.

Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens NM, Gagnon J, Arlaud GJ.

Biochemistry. 1997 May 27;36(21):6270-82.


Synthesis of classical pathway complement components by chondrocytes.

Bradley K, North J, Saunders D, Schwaeble W, Jeziorska M, Woolley DE, Whaley K.

Immunology. 1996 Aug;88(4):648-56.


Expression of the complement classical pathway by human glioma in culture. A model for complement expression by nerve cells.

Gasque P, Ischenko A, Legoedec J, Mauger C, Schouft MT, Fontaine M.

J Biol Chem. 1993 Nov 25;268(33):25068-74.


A common amino acid polymorphism in complement component C1R.

Nöthen MM, Dewald G.

Hum Mol Genet. 1994 Jan;3(1):217. No abstract available.


Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r.

Arlaud GJ, Gagnon J.

Biochemistry. 1983 Apr 12;22(8):1758-64.


Nucleotide sequence of the cDNA coding for human complement C1r.

Leytus SP, Kurachi K, Sakariassen KS, Davie EW.

Biochemistry. 1986 Aug 26;25(17):4855-63.


Identification of erythro-beta-hydroxyasparagine in the EGF-like domain of human C1r.

Arlaud GJ, Van Dorsselaer A, Bell A, Mancini M, Aude C, Gagnon J.

FEBS Lett. 1987 Sep 28;222(1):129-34.


Familial deficiency of two subunits of the first component of complement. C1r and C1s associated with a lupus erythematosus-like disease.

Lee SL, Wallace SL, Barone R, Blum L, Chase PH.

Arthritis Rheum. 1978 Nov-Dec;21(8):958-67.


Periodontal Ehlers-Danlos Syndrome Is Caused by Mutations in C1R and C1S, which Encode Subcomponents C1r and C1s of Complement.

Kapferer-Seebacher I, Pepin M, Werner R, Aitman TJ, Nordgren A, Stoiber H, Thielens N, Gaboriaud C, Amberger A, Schossig A, Gruber R, Giunta C, Bamshad M, Björck E, Chen C, Chitayat D, Dorschner M, Schmitt-Egenolf M, Hale CJ, Hanna D, Hennies HC, Heiss-Kisielewsky I, Lindstrand A, Lundberg P, Mitchell AL, Nickerson DA, Reinstein E, Rohrbach M, Romani N, Schmuth M, Silver R, Taylan F, Vandersteen A, Vandrovcova J, Weerakkody R, Yang M, Pope FM; Molecular Basis of Periodontal EDS Consortium, Byers PH, Zschocke J.

Am J Hum Genet. 2016 Nov 3;99(5):1005-1014. doi: 10.1016/j.ajhg.2016.08.019. Epub 2016 Oct 13.

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