KEGG   PATHWAY: ko05322
Entry
ko05322                     Pathway                                
Name
Systemic lupus erythematosus
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
ko05322  Systemic lupus erythematosus
ko05322

Disease
H00080  Systemic lupus erythematosus
Orthology
K01319  CTSG; cathepsin G [EC:3.4.21.20]
K01327  ELANE; leukocyte elastase [EC:3.4.21.37]
K01330  C1R; complement component 1, r subcomponent [EC:3.4.21.41]
K01331  C1S; complement component 1, s subcomponent [EC:3.4.21.42]
K01332  C2; complement component 2 [EC:3.4.21.43]
K03156  TNF, TNFA; tumor necrosis factor superfamily, member 2
K03160  TNFRSF5, CD40; tumor necrosis factor receptor superfamily member 5
K03161  TNFSF5, CD40L, CD154; tumor necrosis factor ligand superfamily member 5
K03986  C1QA; complement C1q subcomponent subunit A
K03987  C1QB; complement C1q subcomponent subunit B
K03988  C1QG; complement C1q subcomponent subunit C
K03989  C4; complement component 4
K03990  C3; complement component 3
K03994  C5; complement component 5
K03995  C6; complement component 6
K03996  C7; complement component 7
K03997  C8A; complement component 8 subunit alpha
K03998  C8B; complement component 8 subunit beta
K03999  C8G; complement component 8 subunit gamma
K04000  C9; complement component 9
K04687  IFNG; interferon gamma
K05209  GRIN2A; glutamate receptor ionotropic, NMDA 2A
K05210  GRIN2B; glutamate receptor ionotropic, NMDA 2B
K05412  CD80; CD80 antigen
K05413  CD86; CD86 antigen
K05443  IL10, CSIF; interleukin 10
K05699  ACTN1_4; actinin alpha 1/4
K06463  FCGR3, CD16; low affinity immunoglobulin gamma Fc receptor III
K06470  CD28; CD28 antigen
K06472  FCGR2A, CD32; low affinity immunoglobulin gamma Fc receptor II-a
K06498  FCGR1A, CD64; high affinity immunoglobulin gamma Fc receptor I
K06752  MHC2; MHC class II antigen
K06856  IGH; immunoglobulin heavy chain
K10651  TRIM21, SSA1; tripartite motif-containing protein 21 [EC:2.3.2.27]
K10784  TRAV; T cell receptor alpha chain V region
K10785  TRBV; T-cell receptor beta chain V region
K11086  SNRPB, SMB; small nuclear ribonucleoprotein B and B'
K11087  SNRPD1, SMD1; small nuclear ribonucleoprotein D1
K11088  SNRPD3, SMD3; small nuclear ribonucleoprotein D3
K11089  TROVE2, SSA2; 60 kDa SS-A/Ro ribonucleoprotein
K11090  LA, SSB; lupus La protein
K11251  H2A; histone H2A
K11252  H2B; histone H2B
K11253  H3; histone H3
K11254  H4; histone H4
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
ko04060  Cytokine-cytokine receptor interaction
ko04514  Cell adhesion molecules
ko04610  Complement and coagulation cascades
ko04612  Antigen processing and presentation
ko04630  JAK-STAT signaling pathway
ko04660  T cell receptor signaling pathway
ko04662  B cell receptor signaling pathway
ko04670  Leukocyte transendothelial migration

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