| Inventor |
|---|
| Pranab K Das |
| SKU: | 154102 |
|---|---|
| Product description: | Can be used as antigen presenting cells. Hyper IgM syndromesÄÂ? is a group ofÄÂ? primary immune deficiency disordersÄÂ? characterised by defectiveÄÂ? CD40ÄÂ? signalling;ÄÂ? viaÄÂ? B cellsÄÂ? affectingÄÂ? class switch recombinationÄÂ? (CSR) andÄÂ? somatic hyper mutation.ÄÂ? ImmunoglobulinÄÂ? (Ig) class switch recombination deficiencies are characterised by elevated serumÄÂ? Immunoglobulin MÄÂ? (IgM) levels and a considerable deficiency inÄÂ? Immunoglobulins GÄÂ? (IgG),ÄÂ? AÄÂ? (IgA) andÄÂ? EÄÂ? (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 1 (X-linked), is characterised by mutations of the CD40LG gene. In this type, T cells cannot tell B cells to switch classes. |
| Alternate name: | TRAP, TNFSF5, TNF-Related Activation Protein, T-Cell Antigen Gp39 |
| Conditional: | No |
| Production details: | B Cells were isolated from PBMCs of CD40L deficient Hyper IgM Syndrome patients using standards methods. B cells were transformed with Epstein-Barr virus. |
| Parental cell line: | B cells from CD40L deficient patient |
| Disease: | Hyper IgM Syndrome type 1 |
| Cat. #: | 154102 |
|---|---|
| Unit size: | 1×10^6 cells / vial |
| Organism: | Human |
| Model: | Immortalised Line |
| Growth properties: | Maintain at 3×105 to 7×105 cells per ml for optimal growth. Replenish growth medium twice per week |
| Format: | Frozen |
|---|---|
| Shipping conditions: | Dry ice |
| Growth medium: | RPMI 1640 medium supplemented with 10% FCS, 10mM HEPES buffer, 50U/ml penicillin and 50 ??g/ml streptomycin |
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