Entry Name Systemic lupus erythematosus - Loxodonta africana (African savanna elephant)
Description Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and certain cytoplasmic components, in association with a diverse array of clinical manifestations. The primary pathological findings in patients with SLE are those of inflammation, vasculitis, immune complex deposition, and vasculopathy. Immune complexes comprising autoantibody and self-antigen is deposited particulary in the renal glomeruli and mediate a systemic inflammatory response by activating complement or via Fc{gamma}R-mediated neutrophil and macrophage activation. Activation of complement (C5) leads to injury both through formation of the membrane attack complex (C5b-9) or by generation of the anaphylatoxin and cell activator C5a. Neutrophils and macrophages cause tissue injury by the release of oxidants and proteases.
Class Human Diseases; Immune diseaseBRITE hierarchy
Pathway map Ortholog table Organism Loxodonta africana (African savanna elephant) [GN:
lav ]
Gene 100664959 C1QA; complement C1q subcomponent subunit A isoform X1 [KO:K03986 ]
100670616 SNRPB; small nuclear ribonucleoprotein-associated proteins B and B' [KO:K11086 ]
100660738 GRIN2B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2B [KO:K05210 ]
100672091 HLA class II histocompatibility antigen, DO alpha chain isoform X1 [KO:K06752 ]
100672379 HLA class II histocompatibility antigen, DM alpha chain [KO:K06752 ]
100664409 HLA class II histocompatibility antigen, DQ alpha 2 chain-like [KO:K06752 ]
100665293 HLA class II histocompatibility antigen, DRB1-4 beta chain-like [KO:K06752 ]
100662176 HLA class II histocompatibility antigen, DP alpha 1 chain-like isoform X4 [KO:K06752 ]
100668794 HLA class II histocompatibility antigen, DQ beta 2 chain-like isoform X2 [KO:K06752 ]
100673529 HLA class II histocompatibility antigen, DO beta chain [KO:K06752 ]
100675728 DLA class II histocompatibility antigen, DR-1 beta chain-like [KO:K06752 ]
100669075 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DQ beta 1 chain-like [KO:K06752 ]
100666146 HLA class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100665860 DLA class II histocompatibility antigen, DR-1 beta chain-like [KO:K06752 ]
100675445 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100667002 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100664981 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100665266 LOW QUALITY PROTEIN: mamu class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100671834 HLA class II histocompatibility antigen, DP beta 1 chain-like [KO:K06752 ]
111748122 SLA class II histocompatibility antigen, DQ haplotype C beta chain-like [KO:K06752 ]
100672669 HLA class II histocompatibility antigen, DM beta chain isoform X1 [KO:K06752 ]
100674958 mamu class II histocompatibility antigen, DR alpha chain [KO:K06752 ]
100665550 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DR alpha chain-like [KO:K06752 ]
100665834 DLA class II histocompatibility antigen, DR-1 beta chain-like [KO:K06752 ]
111748125 HLA class II histocompatibility antigen, DP beta 1 chain-like [KO:K06752 ]
100671798 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DP alpha 1 chain [KO:K06752 ]
100673824 HLA class II histocompatibility antigen, DQ beta 2 chain-like [KO:K06752 ]
100659676 DLA class II histocompatibility antigen, DR-1 beta chain-like [KO:K06752 ]
100656594 CD86; T-lymphocyte activation antigen CD86 isoform X1 [KO:K05413 ]
100661033 CD28; T-cell-specific surface glycoprotein CD28 isoform X1 [KO:K06470 ]
100666178 CD40; tumor necrosis factor receptor superfamily member 5 [KO:K03160 ]
100654974 FCGR1A; high affinity immunoglobulin gamma Fc receptor I [KO:K06498 ]
100654406 FCGR1B; high affinity immunoglobulin gamma Fc receptor IB isoform X1 [KO:K06498 ]
104846329 FCGR2A; low affinity immunoglobulin gamma Fc region receptor II-a isoform X2 [KO:K06472 ]
104846346 low affinity immunoglobulin gamma Fc region receptor III-A-like [KO:K06463 ]
Compound Reference Authors Rahman A, Isenberg DA.
Title Systemic lupus erythematosus.
Journal Reference Authors Mok CC, Lau CS.
Title Pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Clatworthy MR, Smith KG.
Title B cells in glomerulonephritis: focus on lupus nephritis.
Journal Reference Authors Couser WG.
Title New insights into mechanisms of immune glomerular injury.
Journal West J Med 160:440-6 (1994)
Reference Authors Cook HT, Botto M.
Title Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Carroll MC.
Title A protective role for innate immunity in systemic lupus erythematosus.
Journal Reference Authors Sturfelt G, Truedsson L.
Title Complement and its breakdown products in SLE.
Journal Reference Authors Lu L, Kaliyaperumal A, Boumpas DT, Datta SK.
Title Major peptide autoepitopes for nucleosome-specific T cells of human lupus.
Journal Reference Authors Zieve GW, Khusial PR.
Title The anti-Sm immune response in autoimmunity and cell biology.
Journal Reference Authors Scofield RH, Farris AD, Horsfall AC, Harley JB.
Title Fine specificity of the autoimmune response to the Ro/SSA and La/SSB ribonucleoproteins.
Journal Reference Authors Hoffman RW.
Title T cells in the pathogenesis of systemic lupus erythematosus.
Journal Related lav04060 Cytokine-cytokine receptor interaction
lav04610 Complement and coagulation cascades
lav04612 Antigen processing and presentation
lav04660 T cell receptor signaling pathway
lav04662 B cell receptor signaling pathway
lav04670 Leukocyte transendothelial migration
KO pathway LinkDB All DBs
