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Mouse Anti-C1R Recombinant Antibody (CBLC018-LY) (CBMAB-C9526-LY)

The product is antibody recognizes C1R. The antibody CBLC018-LY immunoassay techniques such as: WB, IP, IF, ELISA.
See all C1R antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBLC018-LY
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Complement C1r
Introduction
C1R (Complement C1r) is a Protein Coding gene. Diseases associated with C1R include Ehlers-Danlos Syndrome, Periodontal Type, 1 and Periodontal Ehlers-Danlos Syndrome. Among its related pathways are Creation of C4 and C2 activators and Complement and coagulation cascades.
Gene Ontology (GO) annotations related to this gene include calcium ion binding and serine-type peptidase activity. An important paralog of this gene is MASP2.
Entrez Gene ID
Human715
Mouse50909
Rat312705
UniProt ID
HumanP00736
MouseQ566I6
RatB5DEH7
Function
C1r B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system.
Biological Process
Complement activation Source: Reactome
Complement activation, classical pathway Source: Reactome
Immune response Source: ProtInc
Innate immune response Source: UniProtKB-KW
Regulation of complement activation Source: Reactome
Zymogen activation Source: CAFA
Cellular Location
Secreted
Involvement in disease
Ehlers-Danlos syndrome, periodontal type, 1 (EDSPD1): A form of Ehlers-Danlos syndrome, a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDSPD1 is characterized by the association of typical features of Ehlers-Danlos syndrome with gingival recession and severe early-onset periodontal disease, leading to premature loss of permanent teeth. EDSPD1 inheritance is autosomal dominant.
PTM
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.

Gaboriaud, C., Rossi, V., & Thielens, N. M. (2021). Structures of the MASP proteases and comparison with complement C1r and C1s. In The Collectin Protein Family and Its Multiple Biological Activities (pp. 73-101). Springer, Cham.

Garrigues, R. J., Powell, A. D., Hammel, M., Skare, J. T., & Garcia, B. L. (2021). A structural basis for inhibition of the complement initiator protease C1r by Lyme disease spirochetes. bioRxiv.

Riihilä, P., Viiklepp, K., Nissinen, L., Farshchian, M., Kallajoki, M., Kivisaari, A., ... & Kähäri, V. M. (2020). Tumour‐cell‐derived complement components C1r and C1s promote growth of cutaneous squamous cell carcinoma. British Journal of Dermatology, 182(3), 658-670.

Fouët, G., Gout, E., Wicker-Planquart, C., Bally, I., De Nardis, C., Dedieu, S., ... & Rossi, V. (2020). Complement C1q Interacts with LRP1 Clusters II and IV Through a Site Close but Different From the Binding Site of Its C1r and C1s-Associated Proteases. Frontiers in immunology, 11, 2706.

Xavier, S., Sahu, R. K., Bontha, S. V., Mas, V., Taylor, R. P., Megyesi, J., ... & Portilla, D. (2019). Complement C1r serine protease contributes to kidney fibrosis. American Journal of Physiology-Renal Physiology, 317(5), F1293-F1304.

Gröbner, R., Kapferer-Seebacher, I., Amberger, A., Redolfi, R., Dalonneau, F., Björck, E., ... & Zschocke, J. (2019). C1R mutations trigger constitutive complement 1 activation in periodontal Ehlers-Danlos syndrome. Frontiers in immunology, 10, 2537.

Gürz, S., Çelik, B., Menteşe, A., & Altay, D. U. (2018). Diagnostic value of signal peptide-Complement C1r/C1s, Uegf, and Bmp1-epidermal growth factor domain-containing protein 1 on serum and tissue samples in non-small cell lung cancer. Turkish Journal of Thoracic and Cardiovascular Surgery, 26(2), 246.

Almitairi, J. O., Girija, U. V., Furze, C. M., Simpson-Gray, X., Badakshi, F., Marshall, J. E., ... & Wallis, R. (2018). Structure of the C1r–C1s interaction of the C1 complex of complement activation. Proceedings of the National Academy of Sciences, 115(4), 768-773.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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