KEGG   PATHWAY: hsa05322Help
Entry
hsa05322                    Pathway                                

Name
Systemic lupus erythematosus - Homo sapiens (human)
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 diseases
BRITE hierarchy
Pathway map
Systemic lupus erythematosus
hsa05322

All organismsOrtholog table
Disease
H00080  
Systemic lupus erythematosus
Organism
Homo sapiens (human) [GN:hsa]
Gene
712  
C1QA; complement C1q A chain [KO:K03986]
713  
C1QB; complement C1q B chain [KO:K03987]
714  
C1QC; complement C1q C chain [KO:K03988]
717  
C2; complement component 2 [KO:K01332] [EC:3.4.21.43]
720  
C4A; complement component 4A (Rodgers blood group) [KO:K03989]
721  
C4B; complement component 4B (Chido blood group) [KO:K03989]
3014  
H2AFX; H2A histone family member X [KO:K11251]
8338  
HIST2H2AC; histone cluster 2, H2ac [KO:K11251]
85235  
HIST1H2AH; histone cluster 1, H2ah [KO:K11251]
221613  
HIST1H2AA; histone cluster 1, H2aa [KO:K11251]
92815  
HIST3H2A; histone cluster 3, H2a [KO:K11251]
83740  
H2AFB3; H2A histone family member B3 [KO:K11251]
3012  
HIST1H2AE; histone cluster 1, H2ae [KO:K11251]
8335  
HIST1H2AB; histone cluster 1, H2ab [KO:K11251]
55506  
H2AFY2; H2A histone family member Y2 [KO:K11251]
9555  
H2AFY; H2A histone family member Y [KO:K11251]
723790  
HIST2H2AA4; histone cluster 2, H2aa4 [KO:K11251]
55766  
H2AFJ; H2A histone family member J [KO:K11251]
474382  
H2AFB1; H2A histone family member B1 [KO:K11251]
8336  
HIST1H2AM; histone cluster 1, H2am [KO:K11251]
8337  
HIST2H2AA3; histone cluster 2, H2aa3 [KO:K11251]
8969  
HIST1H2AG; histone cluster 1, H2ag [KO:K11251]
317772  
HIST2H2AB; histone cluster 2, H2ab [KO:K11251]
94239  
H2AFV; H2A histone family member V [KO:K11251]
3013  
HIST1H2AD; histone cluster 1, H2ad [KO:K11251]
3015  
H2AFZ; H2A histone family member Z [KO:K11251]
8330  
HIST1H2AK; histone cluster 1, H2ak [KO:K11251]
8334  
HIST1H2AC; histone cluster 1, H2ac [KO:K11251]
8329  
HIST1H2AI; histone cluster 1, H2ai [KO:K11251]
8331  
HIST1H2AJ; histone cluster 1, H2aj [KO:K11251]
8332  
HIST1H2AL; histone cluster 1, H2al [KO:K11251]
474381  
H2AFB2; H2A histone family member B2 [KO:K11251]
8341  
HIST1H2BN; histone cluster 1, H2bn [KO:K11252]
8345  
HIST1H2BH; histone cluster 1, H2bh [KO:K11252]
8342  
HIST1H2BM; histone cluster 1, H2bm [KO:K11252]
158983  
H2BFWT; H2B histone family member W, testis specific [KO:K11252]
255626  
HIST1H2BA; histone cluster 1, H2ba [KO:K11252]
440689  
HIST2H2BF; histone cluster 2, H2bf [KO:K11252]
8348  
HIST1H2BO; histone cluster 1, H2bo [KO:K11252]
3018  
HIST1H2BB; histone cluster 1, H2bb [KO:K11252]
8339  
HIST1H2BG; histone cluster 1, H2bg [KO:K11252]
8340  
HIST1H2BL; histone cluster 1, H2bl [KO:K11252]
8347  
HIST1H2BC; histone cluster 1, H2bc [KO:K11252]
3017  
HIST1H2BD; histone cluster 1, H2bd [KO:K11252]
8344  
HIST1H2BE; histone cluster 1, H2be [KO:K11252]
8346  
HIST1H2BI; histone cluster 1, H2bi [KO:K11252]
8349  
HIST2H2BE; histone cluster 2, H2be [KO:K11252]
8970  
HIST1H2BJ; histone cluster 1, H2bj [KO:K11252]
85236  
HIST1H2BK; histone cluster 1, H2bk [KO:K11252]
128312  
HIST3H2BB; histone cluster 3, H2bb [KO:K11252]
8343  
HIST1H2BF; histone cluster 1, H2bf [KO:K11252]
286436  
H2BFM; H2B histone family member M [KO:K11252]
440093  
H3F3C; H3 histone, family 3C [KO:K11253]
3021  
H3F3B; H3 histone, family 3B [KO:K11253]
8351  
HIST1H3D; histone cluster 1, H3d [KO:K11253]
8352  
HIST1H3C; histone cluster 1, H3c [KO:K11253]
8350  
HIST1H3A; histone cluster 1, H3a [KO:K11253]
3020  
H3F3A; H3 histone, family 3A [KO:K11253]
8290  
HIST3H3; histone cluster 3, H3 [KO:K11253]
126961  
HIST2H3C; histone cluster 2, H3c [KO:K11253]
333932  
HIST2H3A; histone cluster 2, H3a [KO:K11253]
653604  
HIST2H3D; histone cluster 2, H3d [KO:K11253]
8353  
HIST1H3E; histone cluster 1, H3e [KO:K11253]
8354  
HIST1H3I; histone cluster 1, H3i [KO:K11253]
8355  
HIST1H3G; histone cluster 1, H3g [KO:K11253]
8356  
HIST1H3J; histone cluster 1, H3j [KO:K11253]
8357  
HIST1H3H; histone cluster 1, H3h [KO:K11253]
8358  
HIST1H3B; histone cluster 1, H3b [KO:K11253]
8968  
HIST1H3F; histone cluster 1, H3f [KO:K11253]
121504  
HIST4H4; histone cluster 4, H4 [KO:K11254]
554313  
HIST2H4B; histone cluster 2, H4b [KO:K11254]
8294  
HIST1H4I; histone cluster 1, H4i [KO:K11254]
8360  
HIST1H4D; histone cluster 1, H4d [KO:K11254]
8361  
HIST1H4F; histone cluster 1, H4f [KO:K11254]
8362  
HIST1H4K; histone cluster 1, H4k [KO:K11254]
8363  
HIST1H4J; histone cluster 1, H4j [KO:K11254]
8364  
HIST1H4C; histone cluster 1, H4c [KO:K11254]
8365  
HIST1H4H; histone cluster 1, H4h [KO:K11254]
8366  
HIST1H4B; histone cluster 1, H4b [KO:K11254]
8367  
HIST1H4E; histone cluster 1, H4e [KO:K11254]
8368  
HIST1H4L; histone cluster 1, H4l [KO:K11254]
8370  
HIST2H4A; histone cluster 2, H4a [KO:K11254]
8359  
HIST1H4A; histone cluster 1, H4a [KO:K11254]
8369  
HIST1H4G; histone cluster 1, H4g [KO:K11254]
6628  
SNRPB; small nuclear ribonucleoprotein polypeptides B and B1 [KO:K11086]
6632  
SNRPD1; small nuclear ribonucleoprotein D1 polypeptide [KO:K11087]
6634  
SNRPD3; small nuclear ribonucleoprotein D3 polypeptide [KO:K11088]
2903  
GRIN2A; glutamate ionotropic receptor NMDA type subunit 2A [KO:K05209]
2904  
GRIN2B; glutamate ionotropic receptor NMDA type subunit 2B [KO:K05210]
6737  
TRIM21; tripartite motif containing 21 [KO:K10651] [EC:2.3.2.27]
6738  
TROVE2; TROVE domain family member 2 [KO:K11089]
6741  
SSB; Sjogren syndrome antigen B [KO:K11090]
87  
ACTN1; actinin alpha 1 [KO:K05699]
88  
ACTN2; actinin alpha 2 [KO:K05699]
89  
ACTN3; actinin alpha 3 (gene/pseudogene) [KO:K05699]
81  
ACTN4; actinin alpha 4 [KO:K05699]
3108  
HLA-DMA; major histocompatibility complex, class II, DM alpha [KO:K06752]
3109  
HLA-DMB; major histocompatibility complex, class II, DM beta [KO:K06752]
3111  
HLA-DOA; major histocompatibility complex, class II, DO alpha [KO:K06752]
3112  
HLA-DOB; major histocompatibility complex, class II, DO beta [KO:K06752]
3113  
HLA-DPA1; major histocompatibility complex, class II, DP alpha 1 [KO:K06752]
3115  
HLA-DPB1; major histocompatibility complex, class II, DP beta 1 [KO:K06752]
3117  
HLA-DQA1; major histocompatibility complex, class II, DQ alpha 1 [KO:K06752]
3118  
HLA-DQA2; major histocompatibility complex, class II, DQ alpha 2 [KO:K06752]
3119  
HLA-DQB1; major histocompatibility complex, class II, DQ beta 1 [KO:K06752]
3122  
HLA-DRA; major histocompatibility complex, class II, DR alpha [KO:K06752]
3123  
HLA-DRB1; major histocompatibility complex, class II, DR beta 1 [KO:K06752]
3125  
HLA-DRB3; major histocompatibility complex, class II, DR beta 3 [KO:K06752]
3126  
HLA-DRB4; major histocompatibility complex, class II, DR beta 4 [KO:K06752]
3127  
HLA-DRB5; major histocompatibility complex, class II, DR beta 5 [KO:K06752]
941  
CD80; CD80 molecule [KO:K05412]
942  
CD86; CD86 molecule [KO:K05413]
940  
CD28; CD28 molecule [KO:K06470]
959  
CD40LG; CD40 ligand [KO:K03161]
958  
CD40; CD40 molecule [KO:K03160]
7124  
TNF; tumor necrosis factor [KO:K03156]
3458  
IFNG; interferon gamma [KO:K04687]
3586  
IL10; interleukin 10 [KO:K05443]
IGH; putative V-set and immunoglobulin domain-containing-like protein IGHV4OR15-8 [KO:K06856]
715  
C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
716  
C1S; complement component 1, s subcomponent [KO:K01331] [EC:3.4.21.42]
718  
C3; complement component 3 [KO:K03990]
727  
C5; complement component 5 [KO:K03994]
729  
C6; complement component 6 [KO:K03995]
730  
C7; complement component 7 [KO:K03996]
731  
C8A; complement component 8 alpha subunit [KO:K03997]
732  
C8B; complement component 8, beta polypeptide [KO:K03998]
733  
C8G; complement component 8, gamma polypeptide [KO:K03999]
735  
C9; complement component 9 [KO:K04000]
1511  
CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
1991  
ELANE; elastase, neutrophil expressed [KO:K01327] [EC:3.4.21.37]
2209  
FCGR1A; Fc fragment of IgG receptor Ia [KO:K06498]
2212  
FCGR2A; Fc fragment of IgG receptor IIa [KO:K06472]
2214  
FCGR3A; Fc fragment of IgG receptor IIIa [KO:K06463]
2215  
FCGR3B; Fc fragment of IgG receptor IIIb [KO:K06463]
 
Compound
C00027  
Hydrogen peroxide
C00434  
Double-stranded DNA
C02737  
Phosphatidylserine
Reference
  Authors
Rahman A, Isenberg DA.
  Title
Systemic lupus erythematosus.
  Journal
N Engl J Med 358:929-39 (2008)
Reference
  Authors
Mok CC, Lau CS.
  Title
Pathogenesis of systemic lupus erythematosus.
  Journal
J Clin Pathol 56:481-90 (2003)
Reference
  Authors
Clatworthy MR, Smith KG.
  Title
B cells in glomerulonephritis: focus on lupus nephritis.
  Journal
Semin Immunopathol 29:337-53 (2007)
Reference
PMID:8048228
  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
Nat Clin Pract Rheumatol 2:330-7 (2006)
Reference
  Authors
Carroll MC.
  Title
A protective role for innate immunity in systemic lupus erythematosus.
  Journal
Nat Rev Immunol 4:825-31 (2004)
Reference
  Authors
Sturfelt G, Truedsson L.
  Title
Complement and its breakdown products in SLE.
  Journal
Rheumatology (Oxford) 44:1227-32 (2005)
Reference
  Authors
Lu L, Kaliyaperumal A, Boumpas DT, Datta SK.
  Title
Major peptide autoepitopes for nucleosome-specific T cells of human lupus.
  Journal
J Clin Invest 104:345-55 (1999)
Reference
  Authors
Zieve GW, Khusial PR.
  Title
The anti-Sm immune response in autoimmunity and cell biology.
  Journal
Autoimmun Rev 2:235-40 (2003)
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
Arthritis Rheum 42:199-209 (1999)
Reference
  Authors
Hoffman RW.
  Title
T cells in the pathogenesis of systemic lupus erythematosus.
  Journal
Clin Immunol 113:4-13 (2004)
KO pathway
 

DBGET integrated database retrieval system