B-LCL-CDG5 Cells
USD$800.00*
Products are shipped frozen on dry ice in cryotubes. Each cryotube typically contains 3 × 106 cells for adherent lines or 5 × 106 cells for suspension lines (refer to the batch CoA for details).
General information
| Description | B-LCL-CDG5 is an EBV-transformed B lymphocyte cell line derived from a patient with PMM2-CDG, a congenital disorder of glycosylation (CDG) caused by mutations in the *PMM2* gene. This disorder impairs the proper synthesis and attachment of glycan structures to glycoproteins and glycolipids, affecting multiple organ systems. The deficiency in phosphomannomutase 2 (PMM2) disrupts the conversion of mannose-6-phosphate to mannose-1-phosphate, a critical step in glycosylation, leading to defects in cellular function and systemic complications. As an EBV-immortalized B cell line, B-LCL-CDG5 serves as a crucial model for studying the biochemical and molecular effects of *PMM2* mutations. This cell line enables researchers to investigate glycosylation defects, PMM2 enzymatic activity, and the cellular consequences of impaired glycosylation. Additionally, it provides a platform for testing potential therapeutic approaches, such as pharmacological chaperones, enzyme enhancement therapies, or substrate supplementation strategies. B-LCL-CDG5, in combination with other CDG patient-derived cell lines, aids in advancing our understanding of PMM2-CDG and the development of targeted treatment options. |
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| Organism | Human |
| Tissue | Peripheral blood |
| Disease | Normal |
| Applications | Genotyping of CDG effects in immune cells, functional testing (e.g. B cell surface antigens), testing of cytotoxic drugs. Mutational analysis, analysis of apoptotic mechanisms, HLA-typing, impact of defective glycosylation of distinct cellular glycoproteins on diverse functions. |
Characteristics
| Gender | Female |
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| Ethnicity | Caucasian |
| Morphology | Round cells |
| Cell type | B lymphocyte |
| Growth properties | Suspension, Cluster |
Regulatory Data
| Citation | B-LCL-CDG5 (Cytion catalog number 302016) |
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| Biosafety level | 2 |
| NCBI_TaxID | 9606 |
Biomolecular Data
| Viruses | Transformant: EBV |
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Handling
| Culture Medium | RPMI 1640, w: 2.0 mM stable Glutamine, w: 2.0 g/L NaHCO3 (Cytion article number 820700a) |
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| Supplements | Supplement the medium with 10% heat-inactivated FBS |
| Subculturing | Maintain cultures by periodically adding or replacing the medium. Initiate cultures with a density of 2 x 105 cells/ml and keep the cell concentration within the range of 1 x 105 to 5 x 105 cells/ml for optimal growth. |
| Fluid renewal | Once the medium colour turned into yellow |
| Post-Thaw Recovery | Medium |
| Freeze medium | As a cryopreservation medium, we use complete growth medium (including FBS) + 10% DMSO for adequate post-thaw viability, or CM-1 (Cytion catalog number 800100), which includes optimized osmoprotectants and metabolic stabilizers to enhance recovery and reduce cryo-induced stress. |
| Thawing and Culturing Cells |
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| Incubation Atmosphere | 37°C, 5% CO2, humidified atmosphere. |
| Shipping Conditions | Cryopreserved cell lines are shipped on dry ice in validated, insulated packaging with sufficient refrigerant to maintain approximately −78 °C throughout transit. On receipt, inspect the container immediately and transfer vials without delay to appropriate storage. |
| Storage Conditions | For long-term preservation, place vials in vapor-phase liquid nitrogen at about −150 to −196 °C. Storage at −80 °C is acceptable only as a short interim step before transfer to liquid nitrogen. |
Quality Control & Molecular Analysis
| Sterility | Mycoplasma contamination is excluded using both PCR-based assays and luminescence-based mycoplasma detection methods. To ensure there is no bacterial, fungal, or yeast contamination, cell cultures are subjected to daily visual inspections. |
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