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Info
Catalogue Number | 154101 |
Parental Line | B cells from CD40 deficient patient |
Synonyms | Tumour Necrosis Factor Receptor Super family Member 5, CD40L Receptor, B Cell Surface Antigen CD40, TNFRSF5 |
Host | Human |
Disease Keywords | Hyper IgM Syndrome type 3 |
Model | Immortalised Line |
Relevance | Hyper IgM syndromes is a group of primary immune deficiency disorders characterised by defective CD40 signalling; via B cells affecting class switch recombination (CSR) and somatic hyper mutation. Immunoglobulin (Ig) class switch recombination deficiencies are characterised by elevated serum Immunoglobulin M (IgM) levels and a considerable deficiency in Immunoglobulins G (IgG), A (IgA) and E (IgE). As a consequence, people with HIGM have decreased concentrations of serum IgG and IgA and normal or elevated IgM, leading to increased susceptibility to infections. Hyper IgM Syndrome type 3 is characterised by mutations of the CD40 gene. In this type, B cells cannot receive the signal from T cells to switch classes |
Production Details | B Cells were isolated from PBMCs of CD40 deficient Hyper IgM Syndrome patients using standards methods. B cells were transformed with Epstein-Barr virus. |
Conditional | No |
Research Area | Cell Type or Organelle Marker, Immunology |
Growth/Phenotype Keywords | Maintain at 3x105 to 7x105 cells per ml for optimal growth. Replenish growth medium twice per week |
Recommended Growing Conditions | RPMI 1640 medium supplemented with 10% FCS, 10mM HEPES buffer, 50U/ml penicillin and 50µg/ml streptomycin |
Notes | Can be used as antigen presenting cells |
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