KEGG   PATHWAY: hsa05322
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 disease
Pathway map
hsa05322  Systemic lupus erythematosus
hsa05322

Disease
H00080  Systemic lupus erythematosus
Organism
Homo sapiens (human) [GN:hsa]
Gene
100293534  C4B_2; complement C4-B-like preproprotein [KO:K03989]
102723407  [KO:K06856]
102724334  [KO:K11252]
110384692  C4A_2; complement C4A (Rodgers blood group)-like preproprotein [KO:K03989]
114483833  H2BK1; histone H2B type 2-K1 [KO:K11252]
115482686  H2AL3; histone H2A-like 3 [KO:K11251]
121504  H4C16; histone H4 [KO:K11254]
126961  H3C14; histone H3.2 [KO:K11253]
128312  H2BC26; histone H2B type 3-B [KO:K11252]
1511  CTSG; cathepsin G preproprotein [KO:K01319] [EC:3.4.21.20]
158983  H2BW1; histone H2B type W-T [KO:K11252]
1991  ELANE; neutrophil elastase preproprotein [KO:K01327] [EC:3.4.21.37]
2209  FCGR1A; high affinity immunoglobulin gamma Fc receptor I isoform 2 precursor [KO:K06498]
2212  FCGR2A; low affinity immunoglobulin gamma Fc region receptor II-a isoform 1 precursor [KO:K06472]
2214  FCGR3A; low affinity immunoglobulin gamma Fc region receptor III-A isoform c precursor [KO:K06463]
2215  FCGR3B; low affinity immunoglobulin gamma Fc region receptor III-B isoform 2 precursor [KO:K06463]
221613  H2AC1; histone H2A type 1-A [KO:K11251]
255626  H2BC1; histone H2B type 1-A [KO:K11252]
286436  H2BW2; histone H2B type F-M [KO:K11252]
2903  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform 1 precursor [KO:K05209]
2904  GRIN2B; glutamate receptor ionotropic, NMDA 2B isoform a precursor [KO:K05210]
3012  H2AC8; histone H2A type 1-B/E [KO:K11251]
3013  H2AC7; histone H2A type 1-D [KO:K11251]
3014  H2AX; histone H2AX [KO:K11251]
3015  H2AZ1; histone H2A.Z [KO:K11251]
3017  H2BC5; histone H2B type 1-D [KO:K11252]
3018  H2BC3; histone H2B type 1-B [KO:K11252]
3020  H3-3A; histone H3.3 [KO:K11253]
3021  H3-3B; histone H3.3 [KO:K11253]
3108  HLA-DMA; HLA class II histocompatibility antigen, DM alpha chain precursor [KO:K06752]
3109  HLA-DMB; HLA class II histocompatibility antigen, DM beta chain precursor [KO:K06752]
3111  HLA-DOA; HLA class II histocompatibility antigen, DO alpha chain precursor [KO:K06752]
3112  HLA-DOB; HLA class II histocompatibility antigen, DO beta chain precursor [KO:K06752]
3113  HLA-DPA1; HLA class II histocompatibility antigen, DP alpha 1 chain precursor [KO:K06752]
3115  HLA-DPB1; HLA class II histocompatibility antigen, DP beta 1 chain precursor [KO:K06752]
3117  HLA-DQA1; HLA class II histocompatibility antigen, DQ alpha 1 chain precursor [KO:K06752]
3118  HLA-DQA2; HLA class II histocompatibility antigen, DQ alpha 2 chain precursor [KO:K06752]
3119  HLA-DQB1; HLA class II histocompatibility antigen, DQ beta 1 chain isoform 2 precursor [KO:K06752]
3120  HLA-DQB2; HLA class II histocompatibility antigen, DQ beta 2 chain isoform 2 precursor [KO:K06752]
3122  HLA-DRA; HLA class II histocompatibility antigen, DR alpha chain precursor [KO:K06752]
3123  HLA-DRB1; major histocompatibility complex, class II, DR beta 1 precursor [KO:K06752]
3125  HLA-DRB3; major histocompatibility complex, class II, DR beta 3 precursor [KO:K06752]
3126  HLA-DRB4; major histocompatibility complex, class II, DR beta 4 precursor [KO:K06752]
3127  HLA-DRB5; major histocompatibility complex, class II, DR beta 5 precursor [KO:K06752]
317772  H2AC21; histone H2A type 2-B [KO:K11251]
333932  H3C15; histone H3.2 [KO:K11253]
340096  H3Y2; histone H3.X [KO:K11253]
3458  IFNG; interferon gamma precursor [KO:K04687]
3586  IL10; interleukin-10 isoform 1 precursor [KO:K05443]
391769  H3Y1; histone H3.Y [KO:K11253]
440093  H3-5; histone H3.3C [KO:K11253]
440686  H3-7; histone H3-7 [KO:K11253]
440689  H2BC18; histone H2B type 2-F isoform a [KO:K11252]
474381  H2AB2; histone H2A-Bbd type 2/3 [KO:K11251]
474382  H2AB1; histone H2A-Bbd type 1 [KO:K11251]
54145  H2BC12L; histone H2B type F-S [KO:K11252]
554313  H4C15; histone H4 [KO:K11254]
55506  MACROH2A2; core histone macro-H2A.2 [KO:K11251]
55766  H2AJ; histone H2A.J [KO:K11251]
653604  H3C13; histone H3.2 [KO:K11253]
6628  SNRPB; small nuclear ribonucleoprotein-associated proteins B and B' isoform B' [KO:K11086]
6632  SNRPD1; small nuclear ribonucleoprotein Sm D1 isoform 1 [KO:K11087]
6634  SNRPD3; small nuclear ribonucleoprotein Sm D3 [KO:K11088]
6737  TRIM21; E3 ubiquitin-protein ligase TRIM21 [KO:K10651] [EC:2.3.2.27]
6738  RO60; RNA-binding protein RO60 isoform 2 [KO:K11089]
6741  SSB; lupus La protein [KO:K11090]
712  C1QA; complement C1q subcomponent subunit A precursor [KO:K03986]
7124  TNF; tumor necrosis factor [KO:K03156]
713  C1QB; complement C1q subcomponent subunit B isoform 1 precursor [KO:K03987]
714  C1QC; complement C1q subcomponent subunit C isoform 1 precursor [KO:K03988]
715  C1R; complement C1r subcomponent isoform 1 preproprotein [KO:K01330] [EC:3.4.21.41]
716  C1S; complement C1s subcomponent isoform 1 preproprotein [KO:K01331] [EC:3.4.21.42]
717  C2; complement C2 isoform 1 preproprotein [KO:K01332] [EC:3.4.21.43]
718  C3; complement C3 preproprotein [KO:K03990]
720  C4A; complement C4-A isoform 1 preproprotein [KO:K03989]
721  C4B; complement C4-B preproprotein [KO:K03989]
723790  H2AC19; histone H2A type 2-A [KO:K11251]
727  C5; complement C5 isoform 1 preproprotein [KO:K03994]
729  C6; complement component C6 precursor [KO:K03995]
730  C7; complement component C7 precursor [KO:K03996]
731  C8A; complement component C8 alpha chain preproprotein [KO:K03997]
732  C8B; complement component C8 beta chain isoform 1 preproprotein [KO:K03998]
733  C8G; complement component C8 gamma chain precursor [KO:K03999]
735  C9; complement component C9 preproprotein [KO:K04000]
81  ACTN4; alpha-actinin-4 isoform 1 [KO:K05699]
8290  H3-4; histone H3.1t [KO:K11253]
8294  H4C9; histone H4 [KO:K11254]
8329  H2AC13; histone H2A type 1 [KO:K11251]
8330  H2AC15; histone H2A type 1 [KO:K11251]
8331  H2AC14; histone cluster 1, H2aj [KO:K11251]
8332  H2AC16; histone H2A type 1 [KO:K11251]
8334  H2AC6; histone H2A type 1-C [KO:K11251]
8335  H2AC4; histone H2A type 1-B/E [KO:K11251]
8336  H2AC17; histone H2A type 1 [KO:K11251]
8337  H2AC18; histone H2A type 2-A [KO:K11251]
8338  H2AC20; histone H2A type 2-C [KO:K11251]
8339  H2BC8; histone H2B type 1-C/E/F/G/I [KO:K11252]
8340  H2BC13; histone H2B type 1-L [KO:K11252]
8341  H2BC15; histone H2B type 1-N [KO:K11252]
8342  H2BC14; histone H2B type 1-M [KO:K11252]
8343  H2BC7; histone H2B type 1-C/E/F/G/I [KO:K11252]
8344  H2BC6; histone H2B type 1-C/E/F/G/I [KO:K11252]
8345  H2BC9; histone H2B type 1-H [KO:K11252]
8346  H2BC10; histone H2B type 1-C/E/F/G/I [KO:K11252]
8347  H2BC4; histone H2B type 1-C/E/F/G/I [KO:K11252]
8348  H2BC17; histone H2B type 1-O [KO:K11252]
8349  H2BC21; histone H2B type 2-E [KO:K11252]
8350  H3C1; histone H3.1 [KO:K11253]
8351  H3C4; histone H3.1 [KO:K11253]
8352  H3C3; histone H3.1 [KO:K11253]
8353  H3C6; histone H3.1 [KO:K11253]
8354  H3C11; histone H3.1 [KO:K11253]
8355  H3C8; histone H3.1 [KO:K11253]
8356  H3C12; histone H3.1 [KO:K11253]
8357  H3C10; histone H3.1 [KO:K11253]
8358  H3C2; histone H3.1 [KO:K11253]
8359  H4C1; histone H4 [KO:K11254]
8360  H4C4; histone H4 [KO:K11254]
8361  H4C6; histone H4 [KO:K11254]
8362  H4C12; histone H4 [KO:K11254]
8363  H4C11; histone H4 [KO:K11254]
8364  H4C3; histone H4 [KO:K11254]
8365  H4C8; histone H4 [KO:K11254]
8366  H4C2; histone H4 [KO:K11254]
8367  H4C5; histone H4 [KO:K11254]
8368  H4C13; histone H4 [KO:K11254]
8369  H4C7; histone H4-like protein type G [KO:K11254]
8370  H4C14; histone H4 [KO:K11254]
83740  H2AB3; histone H2A-Bbd type 2/3 [KO:K11251]
85235  H2AC12; histone H2A type 1-H [KO:K11251]
85236  H2BC12; histone H2B type 1-K [KO:K11252]
87  ACTN1; alpha-actinin-1 isoform b [KO:K05699]
8968  H3C7; histone H3.1 [KO:K11253]
8969  H2AC11; histone H2A type 1 [KO:K11251]
8970  H2BC11; histone H2B type 1-J [KO:K11252]
92815  H2AC25; histone H2A type 3 [KO:K11251]
940  CD28; T-cell-specific surface glycoprotein CD28 isoform 1 precursor [KO:K06470]
941  CD80; T-lymphocyte activation antigen CD80 precursor [KO:K05412]
942  CD86; T-lymphocyte activation antigen CD86 isoform 1 precursor [KO:K05413]
94239  H2AZ2; histone H2A.V isoform 1 [KO:K11251]
9555  MACROH2A1; core histone macro-H2A.1 isoform 1 [KO:K11251]
958  CD40; tumor necrosis factor receptor superfamily member 5 isoform 1 precursor [KO:K03160]
959  CD40LG; CD40 ligand [KO:K03161]
Compound
C00027  Hydrogen peroxide
C00434  Double-stranded DNA
C02737  Phosphatidylserine
Reference
PMID:18305268
  Authors
Rahman A, Isenberg DA.
  Title
Systemic lupus erythematosus.
  Journal
N Engl J Med 358:929-39 (2008)
DOI:10.1056/NEJMra071297
Reference
PMID:12835292
  Authors
Mok CC, Lau CS.
  Title
Pathogenesis of systemic lupus erythematosus.
  Journal
J Clin Pathol 56:481-90 (2003)
DOI:10.1136/jcp.56.7.481
Reference
PMID:17943287
  Authors
Clatworthy MR, Smith KG.
  Title
B cells in glomerulonephritis: focus on lupus nephritis.
  Journal
Semin Immunopathol 29:337-53 (2007)
DOI:10.1007/s00281-007-0092-1
Reference
PMID:8048228
  Authors
Couser WG.
  Title
New insights into mechanisms of immune glomerular injury.
  Journal
West J Med 160:440-6 (1994)
Reference
PMID:16932712
  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)
DOI:10.1038/ncprheum0191
Reference
PMID:15459673
  Authors
Carroll MC.
  Title
A protective role for innate immunity in systemic lupus erythematosus.
  Journal
Nat Rev Immunol 4:825-31 (2004)
DOI:10.1038/nri1456
Reference
PMID:15972354
  Authors
Sturfelt G, Truedsson L.
  Title
Complement and its breakdown products in SLE.
  Journal
Rheumatology (Oxford) 44:1227-32 (2005)
DOI:10.1093/rheumatology/keh719
Reference
PMID:10430616
  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)
DOI:10.1172/JCI6801
Reference
PMID:12965173
  Authors
Zieve GW, Khusial PR.
  Title
The anti-Sm immune response in autoimmunity and cell biology.
  Journal
Autoimmun Rev 2:235-40 (2003)
DOI:10.1016/S1568-9972(03)00018-1
Reference
PMID:10025913
  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)
DOI:10.1002/1529-0131(199902)42:2<199::AID-ANR1>3.0.CO;2-1
Reference
PMID:15380523
  Authors
Hoffman RW.
  Title
T cells in the pathogenesis of systemic lupus erythematosus.
  Journal
Clin Immunol 113:4-13 (2004)
DOI:10.1016/j.clim.2004.05.001
Related
pathway
hsa04060  Cytokine-cytokine receptor interaction
hsa04514  Cell adhesion molecule (CAM) interaction
hsa04610  Complement and coagulation cascades
hsa04612  Antigen processing and presentation
hsa04630  JAK-STAT signaling pathway
hsa04660  T cell receptor signaling pathway
hsa04662  B cell receptor signaling pathway
hsa04670  Leukocyte transendothelial migration
KO pathway
ko05322   

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