Gene and Protein Information
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	-	705	12p13.31	C1R	complement C1r
Mouse	-	707	6 F2	C1ra	complement component 1, r subcomponent A
Rat	-	707	4q42	C1r	complement C1r

Previous and Unofficial Names

complement C1r-A subcomponent | complement component 1, r subcomponent A | complement component 1

Database Links
Specialist databases
MEROPS	S01.192 (Hs)
Other databases
Alphafold	P00736 (Hs), Q8CG16 (Mm)
BRENDA	3.4.21.41
CATH/Gene3D	2.60.120.290
ChEMBL Target	CHEMBL4611 (Hs)
Ensembl Gene	ENSG00000159403 (Hs), ENSMUSG00000055172 (Mm), ENSRNOG00000011796 (Rn)
Entrez Gene	715 (Hs), 50909 (Mm), 312705 (Rn)
Human Protein Atlas	ENSG00000159403 (Hs)
KEGG Enzyme	3.4.21.41
KEGG Gene	hsa:715 (Hs), mmu:50909 (Mm), rno:312705 (Rn)
OMIM	613785 (Hs)
Orphanet	ORPHA360362 (Hs)
Pharos	P00736 (Hs)
RefSeq Nucleotide	NM_001733 (Hs), NM_023143 (Mm), NM_001134555 (Rn)
RefSeq Protein	NP_001724 (Hs), NP_075632 (Mm), NP_001128027 (Rn)
UniProtKB	P00736 (Hs), Q8CG16 (Mm)
Wikipedia	C1R (Hs)

Enzyme Reaction
EC Number: 3.4.21.41

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference nafamostat Hs Inhibition 4.9 pIC50 1 ⤷ pIC50 4.9 (IC50 1.2x10-5 M) [1]

Immunopharmacology Comments

A protease component of the C1-complex of the classical complement pathway.

Immuno Process Associations

Immuno Process:  Inflammation

Immuno Process:  T cell (activation)

Immuno Process:  B cell (activation)

Immuno Process:  Immune regulation

Clinically-Relevant Mutations and Pathophysiology
Disease:  Complement component C1r/C1s deficiency Synonyms: Immunodeficiency due to an early component of complement deficiency [Orphanet: ORPHA169147] OMIM: 216950 Orphanet: ORPHA169147