KEGG    Network variation - Complement cascade
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ENTRYnt06513
NameComplement cascade
CategoryPathway view; Immune system
Pathwayhsa04610 Complement and coagulation cascades
DiseaseH00102 Classic complement pathway component defects
H00105 Mannose-binding lectin pathway component defects
H00104 Alternative complement pathway component defects
H00103 Late complement pathway defects
H00106 Complement regulatory protein defects
Display drug-target relation   disease type
N01487      [C4,C2]((C1QA,C1QB,C1QC)+..[C4b,C2a](C4b+C2a)
    C4a deficiency   C4A*
    C4b deficiency   C4B*
    C2 deficiency/ARMD14   C2*
    C1qa deficiency     C1QA*
    C1qb deficiency     C1QB*
    C1qc deficiency     C1QC*
    EDSPD1     C1R*
    C1s deficiency/EDSPD2     C1S*
N01314    SARS-CoV-2 S(IgG,IgM)((C1QA,C1QB,C1QC)+..
N00213    KSHV     KCPC4b
N01491    [C4,C2]((MBL2,COLEC10/11,..[C4b,C2a](C4b+C2a)
    LCAPD1   MBL2*
    LCAPD2   MASP2*
    LCAPD3   FCN3*
    3MC1   MASP1*
    3MC2   COLEC11*
    3MC3   COLEC10*
N01312    SARS-CoV-2 S(MBL2+MASP1/2)
N01489    C3(C4b+C2a)C3b(C4b+C2a+C3b)
    C3 deficiency/ARMD9/AHUS5 C3*
N00589    HSV   GCC3b
N01493    C3C3H2O(C3H2O+CFB)CFD(C3H2O+Bb)
    CFB deficiency/ARMD14/AHUS4     CFB*
          CFD*
N01494          C3((C3H2O+Bb),(C3b+B..C3b(C3b+CFB)CFD(C3b+Bb)((C3b+Bb)+CFP)((C3b+Bb+C3b)+CFP)
    CFP deficiency                   CFP*
 D1
 |
      
N01490    C5((C4b+C2a+C3b),(C3..C5b(C5b+C6+C7+C8+C9)
    C5D C5*
    C6D       C6*
    C7D       C7*
    C8D1       C8A*
    C8D2       C8B*
    C9D/ARMD15       C9*
N01505        (CD59,CLU,VTN)(C5b+C6+C7+C8+C9)
    CD59 deficiency     CD59*
N01495    (C4b+C2a)C4BPC4b(CFI+C4BP)(C4c,C4d)
N01527    KSHV     C4b(CFI+KCP)C4d
N01496    (C3b+Bb)(CFH,(CD55+CR1))C3b(CFI+(CFH,CR1,CD46..iC3b(CFI+CR1)C3dgPlasminC3d
    CFH deficiency/C3G1/BLD/ARMD4/AHUS1   CFH*
    CD55 deficiency   CD55*
    CFI deficiency/C3G2/ARMD13/AHUS3       CFI*
    AHUS2       CD46*
N01528    KSHV     C3b(CFI+KCP)iC3b(CFI+KCP)C3dg
N01504    CFHRCFH
    ARMD1/AHUS1 CFHR1*
    ARMD1/AHUS1 CFHR3*
    C3G3 CFHR5*
N01520    SERPING1(F11a,F12a,Kallikr..
    C1NH deficiency SERPING1*

Disease nameDisease category
C4a deficiencyH00102Classic complement pathway component defectsPrimary immunodeficiency
C4b deficiencyH00102Classic complement pathway component defectsPrimary immunodeficiency
C2 deficiency/ARMD14H00102Classic complement pathway component defectsPrimary immunodeficiency
H00821Age-related macular degenerationNervous system disease
C1qa deficiencyH00102Classic complement pathway component defectsPrimary immunodeficiency
C1qb deficiencyH00102Classic complement pathway component defectsPrimary immunodeficiency
C1qc deficiencyH00102Classic complement pathway component defectsPrimary immunodeficiency
EDSPD1H02240Ehlers-Danlos syndrome periodontal typeCongenital malformation
C1s deficiency/EDSPD2H00102Classic complement pathway component defectsPrimary immunodeficiency
H02240Ehlers-Danlos syndrome periodontal typeCongenital malformation
SARS-CoV-2H02398COVID-19Viral infectious disease
KSHVH00041Kaposi sarcomaCancer
LCAPD1H00105Mannose-binding lectin pathway component defectsPrimary immunodeficiency
LCAPD2H00105Mannose-binding lectin pathway component defectsPrimary immunodeficiency
LCAPD3H00105Mannose-binding lectin pathway component defectsPrimary immunodeficiency
3MC1H018873MC syndromeCongenital malformation
3MC2H018873MC syndromeCongenital malformation
3MC3H018873MC syndromeCongenital malformation
C3 deficiency/ARMD9/AHUS5H00102Classic complement pathway component defectsPrimary immunodeficiency
H00821Age-related macular degenerationNervous system disease
H01434Atypical hemolytic uremic syndromeHematologic disease
HSVH00365Herpes simplex virus infectionViral infectious disease
CFB deficiency/ARMD14/AHUS4H00104Alternative complement pathway component defectsPrimary immunodeficiency
H00821Age-related macular degenerationNervous system disease
H01434Atypical hemolytic uremic syndromeHematologic disease
CFP deficiencyH00104Alternative complement pathway component defectsPrimary immunodeficiency
C5DH00103Late complement pathway defectsPrimary immunodeficiency
C6DH00103Late complement pathway defectsPrimary immunodeficiency
C7DH00103Late complement pathway defectsPrimary immunodeficiency
C8D1H00103Late complement pathway defectsPrimary immunodeficiency
C8D2H00103Late complement pathway defectsPrimary immunodeficiency
C9D/ARMD15H00103Late complement pathway defectsPrimary immunodeficiency
H00821Age-related macular degenerationNervous system disease
CD59 deficiencyH00106Complement regulatory protein defectsPrimary immunodeficiency
CFH deficiency/C3G1/BLD/ARMD4/AHUS1H00104Alternative complement pathway component defectsPrimary immunodeficiency
H02579C3 glomerulopathyImmune system disease
H02108Basal laminar drusenNervous system disease
H00821Age-related macular degenerationNervous system disease
H01434Atypical hemolytic uremic syndromeHematologic disease
CD55 deficiencyH00106Complement regulatory protein defectsPrimary immunodeficiency
CFI deficiency/C3G2/ARMD13/AHUS3H00106Complement regulatory protein defectsPrimary immunodeficiency
H02579C3 glomerulopathyImmune system disease
H00821Age-related macular degenerationNervous system disease
H01434Atypical hemolytic uremic syndromeHematologic disease
AHUS2H01434Atypical hemolytic uremic syndromeHematologic disease
ARMD1/AHUS1H00821Age-related macular degenerationNervous system disease
H01434Atypical hemolytic uremic syndromeHematologic disease
C3G3H02579C3 glomerulopathyImmune system disease
C1NH deficiencyH00106Complement regulatory protein defectsPrimary immunodeficiency


Drug name
D1D11696Crovalimab (USAN/INN)