B6-H11-hBDCA2 (hCLEC4C) Mouse
Product Name
B6-H11-hBDCA2 (hCLEC4C) Mouse
Product ID
C001693
Strain Name
C57BL/6NCya-Igs2em1(hCLEC4C)/Cya
Backgroud
C57BL/6NCya
Status
When using this mouse strain in a publication, please cite “B6-H11-hBDCA2 (hCLEC4C) Mouse (Catalog C001693) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
CLEC4C
Gene Alias
DLEC, HECL, BDCA2, CD303, BDCA-2, CLECSF7, CLECSF11, PRO34150
NCBI ID
Chromosome
Chr 12 (Human)
MGI ID
Datasheet
Strain Description
The CLEC4C gene, also known as BDCA-2 or CD303, encodes a type II transmembrane C-type lectin receptor predominantly expressed by plasmacytoid dendritic cells (pDCs) [1]. This receptor plays a critical role in pDC biology and serves as a key marker for this cell type [2]. The CLEC4C protein, featuring a carbohydrate recognition domain, is implicated in the capture and subsequent processing of antigens, potentially through the recognition of specific glycans and immunoglobulin G [1]. Functionally, CLEC4C acts as a signaling receptor within pDCs, and its engagement can negatively regulate the production of type I interferons, thereby modulating immune responses [2]. Notably, dysregulation of CLEC4C expression and pDC function has been associated with the pathogenesis of autoimmune disorders, including systemic lupus erythematosus (SLE), as well as in the context of certain hematological malignancies [3]. Litifilimab is a monoclonal antibody that targets CLEC4C and is under investigation for the treatment of SLE and other interferonopathies [4].
B6-H11-hCLEC4C mice are humanized models generated by gene editing technology, in which the human CLEC4C genomic DNA was inserted at the H11 safe harbor. This modification does not affect the expression of the mouse homologous gene Clec4b1. This model can be used to study the pathological mechanisms and therapeutic methods of autoimmune disorders and hematological malignancies, as well as the screening and development of CLEC4C-targeted drugs, and preclinical efficacy and safety evaluations.
Reference
Boiocchi L, Lonardi S, Vermi W, Fisogni S, Facchetti F. BDCA-2 (CD303): a highly specific marker for normal and neoplastic plasmacytoid dendritic cells. Blood. 2013 Jul 11;122(2):296-7.
Tsagarakis NJ, Paterakis G. Dendritic Cell Leukemia: a Review. Curr Oncol Rep. 2020 May 15;22(6):55.
Wilson NR, Bover L, Konopleva M, Han L, Neelapu S, Pemmaraju N. CD303 (BDCA-2) - a potential novel target for therapy in hematologic malignancies. Leuk Lymphoma. 2022 Jan;63(1):19-30.
Cho YM, Furie R. The development of litifilimab (BIIB 059) for cutaneous and systemic lupus erythematosus. Immunotherapy. 2024 Jan;16(1):15-20.
Strain Strategy
The "Human CLEC4C Genomic DNA (~5 kb upstream of ATG start codon to ~0.5 kb downstream of 3'UTR)" cassette was inserted into H11 locus (Approx 0.7 kb upstream of the Eif4enif1 gene and 4.5 kb downstream of the Drg1 gene).
Figure 1. Gene editing strategy of B6-H11-hCLEC4C mice.
Application Area
Screening, development, and preclinical efficacy evaluation of CLEC4C-targeted drugs；
Study of pathological mechanisms and therapeutic methods for autoimmune disorders；
Study of pathological mechanisms and therapeutic methods for hematological malignancies.
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