Table 1 Structures, select interacting partners, and cellular functions of human caspases and their disease associations.

From: Caspases: structural and molecular mechanisms and functions in cell death, innate immunity, and disease

Caspase	PDB entry^a	Select interacting partner^b	Cellular function	Disease^c	Disease-associating mutation^d	Inhibitor	Reference
CASP1	1IBC,1ICE,1RWK,1RWM,1RWN,1RWO,1RWP,1RWV,1RWW,1RWX,1SC1,1SC3,1SC4,2FQQ,2H48,2H4W,2H4Y,2H51,2H54,2HBQ,2HBR,2HBY,2HBZ,5MMV,5MTK,6PZP,1BMQ,3D6F,3D6H,3D6M,3NS7,6BZ9,6F6R,3E4C,5FNA,8WRA,6VIE,6KN0,8SV1,7KEU	ARID4B,BRCA1,CARD16,FMN2,GAB1,GSDMD,IFI16,IL1B,KIF11,KIF3A,NLRC4,PI4KB,PLCL2,PYCARD,RNF31,VAC14	Pyroptosis,PANoptosis,Metabolism	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric), Osteoporosis, Parkinson (late onset), Ataxia-telangiectasia, Multiple sclerosis, Rheumatoid arthritis, Retinitis pigmentosa, Lynch syndrome, Pulmonary hypertension, Cone-Rod dystrophy, Sickle cell, Cardio- and nephrological diseases, Schizophrenia, Familial Mediterranean fever, Asthma, Chronic lymphocytic leukemia	K37Q, V61I, C72F	Minocycline, Pralnacasan, LAX-101, Emricasan, VX-765, Z-VAD, VX-166, Belnacasan	^{11,20,262,269,270}
CASP2	3R5J,3R6G,3R6L,3R7B,3R7N,3R7S,1PYO,2P2C,3RJM,6GKF,6GKG,6S9K,6SAD,6Y8B,6Y8D	BBOX1,BID,BIRC2,CASP3,CASP8,CIB1,CRADD,EPN3,HNRNPK,PIDD1,RFXANK,TRAF1,TRAF2,TRAF3	Cell cycle,Autophagy,Genome stability,Tumorigenesis,Tumor suppression,Aging,Apoptosis	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Head and neck, Brain), Familial Alzheimer disease, Osteoporosis, Ataxia-telangiectasia, Ischemic stroke, Multiple sclerosis, Lynch syndrome, Neurofibromatosis type I, Lung cancer susceptibility, Myeloma, Multiple leukemia, Chronic lymphocytic squamous cell carcinoma	R234G^lb, R265^lb	Emricasan, Z-VAD, VX-166	^11,271
CASP3	1GFW,1NME,1PAU,1RE1,1RHJ,1RHK,1RHM,1RHQ,1RHR,1RHU,2C1E,2C2K,2C2M,2C2O,2CDR,2CJX,2CJY,2CNK,2CNL,2CNN,2CNO,2DKO,2H5I,2H5J,2H65,2XYG,2XYH,2XYP,2XZD,2XZT,2Y0B,3EDQ,3GJQ,3GJR,3GJS,3GJT,3KJF,4DCJ,4DCO,4DCP,5IC4,6BDV,6BFJ,6BFK,6BFL,6BFO,6BG0,6BG1,6BG4,6BGK,6BGQ,6BGR,6BGS,6BH9,6BHA,6CKZ,6CL0,6X8I,6X8K,7RN7,7RN8,7RN9,7RNA,7RNB,7RNC,7RND,7RNE,7RNF,7RNG,7SEO,7USO,7USP,7USQ,1CP3,1NMQ,1NMS,1QX3,2J30,2J31,2J32,2J33,3H0E,3ITN,3PCX,3PD0,3PD1,4EHA,4EHD,4EHF,4EHH,4EHK,4EHL,4EHN,4JJE,4JQY,4JQZ,4JR0,4PRY,4PS0,4QTX,4QTY,4QU0,4QU5,4QU9,4QUA,4QUB,4QUD,4QUJ,4QUL,5I9B,5IAJ,5IAK,5IAS,5IBC,5IBP,3DEH,3DEI,3DEJ,3DEK,4QU8,4QUE,4QUG,4QUH,4QUI,5I9T,5IAB,5IAE,5IAG,5IAN,5IAR,5IBR,1I3O,7XN4,7XN5,7XN6	XIAP,PARP1,BIRC2,CASP9,CDKN1A,CRYAB,MDM2,BIRC5,BIRC6,CASP6,CASP8,CFLAR,HCLS1,MAPT,AFP,ARNT,BID,BIRC3,BIRC7,CASP2,CTTN	Apoptosis, PANoptosis, Pyroptosis, Autophagy, Stem cell and neural differentiation	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Esophageal, Endometrial, Brain), Type 1 and 2 diabetes mellitus, Systemic lupus erythematosus, Familial Alzheimer disease, Amyotrophic lateral sclerosis, Hypertension, Acute myeloid leukemia, Crohn’s disease, Age-related macular degeneration, Osteoporosis, Parkinson disease (late onset), Ataxia-telangiectasia, Complementation group Fanconi anemia, Ischemic stroke, Rheumatoid arthritis, Familial meningioma, Duchenne muscular dystrophy, Multiple sclerosis, Retinitis pigmentosa, Nonpapillary renal cell carcinoma, Malaria, Cystic fibrosis, Gastrointestinal stromal tumor, Neural tube defects, Cone-Rod dystrophy, Leptin deficiency, Interstitial lung disease, Medulloblastoma and retinoblastoma, Dilated cardiomyopathy, Schizophrenia, Nephrological diseases, Head and neck squamous cell carcinoma, Lung cancer susceptibility, Autism spectrum disorder, Chronic lymphocytic leukemia, Multiple myeloma, Ulcerative colitis, Tuberous sclerosis, Asthma, Tatton-Brown-Rahman syndrome, Familial adenomatous polyposis	E25K^lb, E34K^lb, R75H^lb, R101H^lb, R110H^lb	Glycyrrhizic acid, Minocycline, Oleandrin, Pamidronic acid, Emricasan, Z-VAD, VX-166, Calpain	^9,11,72,100
CASP4	6NRY,7WR0,7WR6,7WR1,6KMZ,8SPB,8J6K,7WR4,7WR5	CASP5	Apoptosis, Pyroptosis, ER stress	Neuroblastoma, Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Esophageal, Brain), Familial Alzheimer disease, Hypertension, Age-related macular degeneration, Parkinson disease (late onset), Complementation group Fanconi anemia, Nonpapillary renal cell carcinoma, Gastrointestinal stromal tumor, Neural tube defects, Cone-Rod dystrophy, Schizophrenia, Lung cancer susceptibility, Multiple myeloma	E228D, E284D, D47N	Incadronic acid, Emricasan, Z-VAD, VX-166	^11,20
CASP5	–	ARHGAP44, CASP4,LACRT	Pyroptosis	Cancer (Colorectal, Lung, Prostate, Breast, Pancreatic, Ovarian, Gastric, Brain, Endometrial), Nonpapillary renal cell carcinoma, Lynch syndrome, Retinoblastoma, Familial Mediterranean fever, Ulcerative colitis	R11H^lb, R24H^lb, E164Q^lb, E240Q^lb, E324Q^lb, E382Q^lb, E395Q^lb		^11,20
CASP6	3P4U,3P45,3QNW,8EG5,8EG6,3NKF,3V6L,4IYR,2WDP,3K7E,3NR2,3OD5,3S70,4EJF,4FXO,4HVA,4N5D,4N6G,4N7J,4N7M,4NBK,4NBL,4NBN,6DEU,6DEV,8F78,8F96,8F98,8F99,8F9A,8F9B,8F9C,8F9D,8FBV,3S8E,3V6M,8F97	CASP3,CASP8,MAPT,STUB1,USP15	Apoptosis, PANoptosis, Synaptic loss and disfunction, B-cell proliferation	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Endometrial, Esophageal, Brain), Familial Alzheimer disease, Amyotrophic lateral sclerosis, Interstitial lung disease, Lung cancer susceptibility, Head and neck squamous cell carcinoma, Multiple myeloma, Chronic lymphocytic leukemia	-	Emricasan, Z-VAD, VX-166	^11,26,272
CASP7	2QLF,3IBC,3IBF,5IC6,6X8J,6X8L,2QL5,2QL7,2QL9,2QLB,2QLJ,3EDR,4JB8,4LSZ,4ZVO,4ZVP,4ZVQ,4ZVR,4ZVS,4ZVT,4ZVU,5K20,6CL1,6CL2,1K86,1K88,1SHJ,1SHL,4FDL,4FEA,4HQ0,4HQR,4JJ8,4JR1,4JR2,5V6U,5V6Z,8DGZ,8DJ3,1F1J,1GQF,3H1P,3R5K,1I51,1I4O,1KMC,7WZS	ATXN3,BIRC2,BIRC3,BIRC5, BIRC6,CASP8,CCT2,CRYAB, FBXW11,HNRNPH1,HSPA5,NFE2L2, PARP1,PTGES3, RB1,SAT1,TRIM25,XIAP	Apoptosis, PANoptosis, Bone development, Dentinogenesis	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Esophageal, Endometrial), Familial Alzheimer disease, Crohn’s disease, Age-related macular degeneration, Parkinson disease (late onset), Type 1 diabetes mellitus, Rheumatoid arthritis, Ischemic stroke, Duchenne muscular dystrophy, Nonpapillary renal cell carcinoma, Retinitis pigmentosa, Osteoarthritis, Chronic nephrological diseases, Chronic lymphocytic leukemia, Head and Neck squamous cell carcinoma, Tatton-Brown-Rahman syndrome, Lung cancer susceptibility	D230E, T244S, D255E, D263E, D288E, D340E	Fica, Emricasan, Incadronic acid, Z-VAD, VX-166	⁹^,11,273
CASP8	1F9E,1QDU,1QTN,2C2Z,3KJN,3KJQ,6X8H,2K7Z,4JJ7,4PRZ,4ZBW,5H31,5H33,6AGW,7LVJ,7LVM,2Y1L,4PS1,5L08,6PX9,1I4E,5JQE,2FUN,3H11,7DEE,8YM4,8YM5,8YM6,8YNK,8YNL,8YNM,8YNN,8YBX,8YD7,8YD8,8YNI	BCAP31,BCL2,BID,BIRC2,BIRC3,CASP2, CASP3,CASP6,CASP7,CASP9,CASP10,CFLAR,CUL3,DEDD,DEDD2,FADD,FAF1, FAS,FASLG,GMEB1,HECTD3,HIP1,IKBKB, IL1B,MAP1LC3B,NLRC4,NOL3,PEA15, PIK3C3,PML,PRDX6,PTEN,PYCARD, RIPK1,RIPK3,RNF34,SQSTM1,SRC, TICAM1,TNF,TNFAIP3,TNFRSF10A, TNFRSF10B,TNFSF10,TRADD, TRAF1,TRAF2,TRAF6,XIAP	Apoptosis, Pyroptosis, PANoptosis, Necroptosis, Lens cell differentiation, Terminal keratinocytes differentiation, Erythrocytes & platelet formation, T & B cell proliferation	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Gastric, Ovarian, Esophageal, Brain, Endometrial), Type 2 diabetes mellitus, Systemic lupus erythematosus, Amyotrophic lateral sclerosis, Familial Alzheimer disease, Acute myeloid leukemia, Crohn’s disease, Age-related macular degeneration, Osteoporosis, Parkinson disease (late onset), Ataxia-telangiectasia, Type 1 diabetes mellitus, Multiple sclerosis, Familial meningioma, Complementation group Fanconi anemia, Rheumatoid arthritis, Ischemic stroke, Nonpapillary renal cell carcinoma, Cystic fibrosis, Type I neurofibromatosis, Pulmonary hypertension, Lynch syndrome, Neural tube defects, Proteasome-associated autoinflammatory syndrome, Cone-Rod dystrophy, Leptin deficiency, Osteoarthritis, Interstitial lung disease, Medulloblastoma, Retinoblastoma, Autoimmune lymphoproliferative syndrome, Asthma, Head and neck squamous cell carcinoma, Lung cancer susceptibility, Chronic lymphocytic leukemia, Multiple myeloma, Ulcerative colitis, Autism spectrum disorder, Tuberous sclerosis, Familial Mediterranean fever, Tatton-Brown-Rahman syndrome, Familial adenomatous polyposis	K14R, D71H, D80H, D86H, D182H, D201H, D216H, D260H, D262H, D270H, D285H, D296H, D302H, D329H, D344H	Bardoxolone, Bryostatin 1, AN-9, Trichostatin A, Oleandrin, Emricasan, Z-VAD, VX-166	^{11,36,69,70,71,102,105,106,148}
CASP9	2AR9,1JXQ,3V3K,3YGS,4RHW,5WVC,1NW9,3D9T,5JUY,5WVE	ABL1,APAF1,ARNT,BCL2L1,BIRC2,BIRC3, BIRC5,BIRC6,BIRC7,BIRC8,CASP3,CASP8, HECTD3,NLRP1,XIAP	Apoptosis, Synaptic loss, Erythropoiesis, Embryonic development & leukemia, Muscle differentiation	Cancer (Colorectal, Lung, Prostate, Breast, Hepatocellular, Pancreatic, Ovarian, Gastric, Esophageal, Endometrial, Brain), Familial Alzheimer disease, Amyotrophic lateral sclerosis, Acute myeloid leukemia, Crohn’s disease, Osteoporosis, Parkinson disease (late onset), Type 1 diabetes mellitus, Ataxia-telangiectasia, Ischemic stroke, Nonpapillary renal cell carcinoma, Neural tube defects, Cone-Rod dystrophy, Osteoarthritis, Medulloblastoma, Schizophrenia, Dilated cardiomyopathy, Head and neck squamous cell carcinoma, Lung cancer susceptibility, Multiple myeloma, Autism spectrum disorder, Chronic lymphocytic leukemia, Ulcerative colitis, Tuberous sclerosis, Tatton-Brown-Rahman syndrome	L23V, A28V, L106V, T216N, T283N, T366N	Pamidronic acid, Emricasan, Z-VAD, VX-166	^11,41,42
CASP10	–	CASP8,CFLAR,FADD,FAS,PRDX6,RIOK3, RNF34,TNFRSF10B,TNFRSF1A,XIAP	-	Cancer (Gastric, Colorectal, Lung, Prostate, Breast, Pancreatic, Ovarian, Brain, Esophageal), Systemic lupus erythematosus, Amyotrophic lateral sclerosis, Crohn’s disease, Rheumatoid arthritis, Pfeiffer syndrome, Lynch syndrome, Immune deficiency disease, Dilated cardiomyopathy, Retinoblastoma, Medulloblastoma, Autoimmune lymphoproliferative syndrome, Chronic lymphocytic leukemia, Asthma, Multiple myeloma, Familial adenomatous polyposis, Ulcerative colitis	K17E, V343I, A347V, V367I, A371V, Y379C, Y403C, V410I, A414V, Y446C, L455I, L479I, L522I	Emricasan, Z-VAD, VX-166	^11,274
CASP12	–	MAGEA3	ER stress	Cancer (Colorectal, Breast, Ovarian, Gastric), Type 2 diabetes mellitus, Malaria, Cardiovascular disease, Tuberous sclerosis	S53N^lb	Emricasan, Z-VAD, VX-166	^275,276
CASP14	–	ALDH3A1,CCNF,FCF1,FTH1,MEOX2, MYC,TBC1D22B,TP53,ZIC1,ZRANB1	Keratinocyte differentiation	Cancer (Prostate, Breast, Pancreatic, Gastric, Brain), Ischemic stroke, Head and neck squamous cell carcinoma, Autism spectrum disorder	L65V R179Q^lb	Emricasan, Z-VAD, VX-166	^277,278,279

^aListed PDB entries as of 11/01/2024.
^bRetrieved from BioGRID v4.4 database (accessed 03/28/2024). Proteins that support at least two experimental evidences were included.
^cRetrieved from MalaCards database (accessed 03/28/2024). Diseases that obtained a minimum of 75 MIFTS score were only included.
^dRetrieved from ClinVar database (accessed 03/28/2024). The superscript “lb” refers to likely benign. Mutations that did not carry the “lb” superscript are either pathogenic, benign, or disease-associating mutation.
^eRetrieved from DrugBank database (accessed 03/28/2024).

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Table 1 Structures, select interacting partners, and cellular functions of human caspases and their disease associations.

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