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ENTRY	nt06513
Name	Complement cascade
Category	Pathway view; Immune system
Pathway	hsa04610 Complement and coagulation cascades
Disease	H00102 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         KCP ⊣ C4b   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     GC ⊣ C3b   N01493    C3 → C3H2O → (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,D2,D3  |             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) − C4BP → C4b − (CFI+C4BP) → (C4c,C4d) N01527    KSHV         C4b − (CFI+KCP) → C4d N01496    (C3b+Bb) − (CFH,(CD55+CR1)) → C3b − (CFI+(CFH,CR1,CD46.. → iC3b − (CFI+CR1) → C3dg − Plasmin → C3d     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    CFHR ⊣ CFH     ARMD1/AHUS1 CFHR1*     ARMD1/AHUS1 CFHR3*     C3G3 CFHR5*   N01520    SERPING1 ⊣ (F11a,F12a,Kallikr..     C1NH deficiency SERPING1*

		Disease name	Disease category
C4a deficiency	H00102	Classic complement pathway component defects	Primary immunodeficiency
C4b deficiency	H00102	Classic complement pathway component defects	Primary immunodeficiency
C2 deficiency/ARMD14	H00102	Classic complement pathway component defects	Primary immunodeficiency
	H00821	Age-related macular degeneration	Nervous system disease
C1qa deficiency	H00102	Classic complement pathway component defects	Primary immunodeficiency
C1qb deficiency	H00102	Classic complement pathway component defects	Primary immunodeficiency
C1qc deficiency	H00102	Classic complement pathway component defects	Primary immunodeficiency
EDSPD1	H02240	Ehlers-Danlos syndrome periodontal type	Congenital malformation
C1s deficiency/EDSPD2	H00102	Classic complement pathway component defects	Primary immunodeficiency
	H02240	Ehlers-Danlos syndrome periodontal type	Congenital malformation
SARS-CoV-2	H02398	COVID-19	Viral infectious disease
KSHV	H00041	Kaposi sarcoma	Cancer
LCAPD1	H00105	Mannose-binding lectin pathway component defects	Primary immunodeficiency
LCAPD2	H00105	Mannose-binding lectin pathway component defects	Primary immunodeficiency
LCAPD3	H00105	Mannose-binding lectin pathway component defects	Primary immunodeficiency
3MC1	H01887	3MC syndrome	Congenital malformation
3MC2	H01887	3MC syndrome	Congenital malformation
3MC3	H01887	3MC syndrome	Congenital malformation
C3 deficiency/ARMD9/AHUS5	H00102	Classic complement pathway component defects	Primary immunodeficiency
	H00821	Age-related macular degeneration	Nervous system disease
	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
HSV	H00365	Herpes simplex virus infection	Viral infectious disease
CFB deficiency/ARMD14/AHUS4	H00104	Alternative complement pathway component defects	Primary immunodeficiency
	H00821	Age-related macular degeneration	Nervous system disease
	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
CFP deficiency	H00104	Alternative complement pathway component defects	Primary immunodeficiency
C5D	H00103	Late complement pathway defects	Primary immunodeficiency
C6D	H00103	Late complement pathway defects	Primary immunodeficiency
C7D	H00103	Late complement pathway defects	Primary immunodeficiency
C8D1	H00103	Late complement pathway defects	Primary immunodeficiency
C8D2	H00103	Late complement pathway defects	Primary immunodeficiency
C9D/ARMD15	H00103	Late complement pathway defects	Primary immunodeficiency
	H00821	Age-related macular degeneration	Nervous system disease
CD59 deficiency	H00106	Complement regulatory protein defects	Primary immunodeficiency
CFH deficiency/C3G1/BLD/ARMD4/AHUS1	H00104	Alternative complement pathway component defects	Primary immunodeficiency
	H02579	C3 glomerulopathy	Immune system disease
	H02108	Basal laminar drusen	Nervous system disease
	H00821	Age-related macular degeneration	Nervous system disease
	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
CD55 deficiency	H00106	Complement regulatory protein defects	Primary immunodeficiency
CFI deficiency/C3G2/ARMD13/AHUS3	H00106	Complement regulatory protein defects	Primary immunodeficiency
	H02579	C3 glomerulopathy	Immune system disease
	H00821	Age-related macular degeneration	Nervous system disease
	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
AHUS2	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
ARMD1/AHUS1	H00821	Age-related macular degeneration	Nervous system disease
	H01434	Atypical hemolytic uremic syndrome	Hematologic disease
C3G3	H02579	C3 glomerulopathy	Immune system disease
C1NH deficiency	H00106	Complement regulatory protein defects	Primary immunodeficiency

		Drug name
D1	D03940	Eculizumab (USAN/INN)
D2	D11054	Ravulizumab (USAN/INN)
D3	D11696	Crovalimab (USAN/INN)