C1S

Synonyms

EC:3.4.21.42, EC 3.4.21, C1 esterase, Complement component 1 subcomponent s, C1S, Complement C1s, Complement Component 1, S Subcomponent, Basic Proline-Rich Peptide IB-1, Complement C1s Subcomponent, EC 3.4.21.42, EDSPD2

Description

Complement component 1s (C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein involved in the complement system. C1s is part of the C1 complex.In humans, it is encoded by the C1S gene.C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.

KO Status

RenMab: F1 (+/-)

Drug Information

Drugs in clinical trials: 4
Latest Research Phase: Phase 3 Clinical

Drug Name

Code

Phase

Company

Indications

Clinical Trials

Sutimlimab

TNT-009, BIVV-009

Phase 3 Clinical

True North Therapeutics

Purpura, Thrombocytopenic, Idiopathic, Anemia, Clostridium Infections, Rejection of organ transplantation, Anemia, Hemolytic, Autoimmune, Pemphigoid, Bullous, Kidney Failure, Chronic

Mirococept

APT-070, APT-070C

Phase 3 Clinical

Adprotech

Myocardial Reperfusion Injury

ANX-005

ANX-005

Phase 3 Clinical

Annexon

Nerve Degeneration, Huntington Disease, Anemia, Hemolytic, Autoimmune, Guillain-Barre Syndrome, Amyotrophic Lateral Sclerosis

BIVV-020

BIVV-020

Phase 2 Clinical

Bioverativ

Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Purpura, Thrombocytopenic, Idiopathic, Anemia, Hemolytic, Autoimmune, Anemia, Hemolytic

CB-4332

CB-4332

Preclinical

Catalyst Biosciences

Atypical Hemolytic Uremic Syndrome, Glomerulonephritis, Membranoproliferative, Glomerulonephritis, Immunologic Deficiency Syndromes

C1-esterase inhibitor (BioGenius)

Pending

Biogenius

Shock, Septic, Sepsis

Recombinant human C1 inhibitor (Santarus)

Pending

Santarus Inc

EZN-3008

EZN-3008

Pending

Enzon Pharmaceuticals, Belrose Pharma

Autoimmune Diseases, Multiple Sclerosis, Angioedemas, Hereditary

References


Title

Authors

Source

Molecular cloning of cDNA for human complement component C1s. The complete amino acid sequence

McKinnon C.M., Carter P.E., Smyth S.J., Dunbar B., Fothergill J.E.,

Eur. J. Biochem. 169:547-553(1987)

Complete cDNA sequence of human complement Cls and close physical linkage of the homologous genes Cls and Clr

Tosi M., Duponchel C., Meo T., Julier C.,

Biochemistry 26:8516-8524(1987)

Human genes for complement components C1r and C1s in a close tail-to-tail arrangement

Kusumoto H., Hirosawa S., Salier J.-P., Hagen F.S., Kurachi K.,

Proc. Natl. Acad. Sci. U.S.A. 85:7307-7311(1988)

The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)

The MGC Project Team,

Genome Res. 14:2121-2127(2004)

Two lineages of mannose-binding lectin-associated serine protease (MASP) in vertebrates

Endo Y., Takahashi M., Nakao M., Saiga H., Sekine H., Matsushita M., Nonaka M., Fujita T.,

J. Immunol. 161:4924-4930(1998)

Complement genes C1r and C1s feature an intronless serine protease domain closely related to haptoglobin

Tosi M., Duponchel C., Meo T., Couture-Tosi E.,

J. Mol. Biol. 208:709-714(1989)

Human complement component C1s. Partial sequence determination of the heavy chain and identification of the peptide bond cleaved during activation

Spycher S.E., Nick H., Rickli E.E.,

Eur. J. Biochem. 156:49-57(1986)

The serine proteinase chain of human complement component C1s. Cyanogen bromide cleavage and N-terminal sequences of the fragments

Carter P.E., Dunbar B., Fothergill J.E.,

Biochem. J. 215:565-571(1983)

Chemical and functional characterization of a fragment of C1-s containing the epidermal growth factor homology region

Thielens N.M., van Dorsselaer A., Gagnon J., Arlaud G.J.,

Biochemistry 29:3570-3578(1990)

Effect of lactoperoxidase-catalyzed iodination on the Ca(2+)-dependent interactions of human C1s. Location of the iodination sites

Illy C., Thielens N.M., Gagnon J., Arlaud G.J.,

Biochemistry 30:7135-7141(1991)