B-LCL-CDG1 cell

SKU:BHC11101674
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Overview
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B-LCL-CDG1 cell is a B lymphocyte cell line derived from Caucasian (Female). It is commonly used as an in vitro model for 2 research. Growth characteristics: Suspension, Cluster, Round cells. Supplied as cryopreserved cells with accompanying batch CoA and quality-control documentation.

Species Human
Disease model Congenital Disorders of Glycosylation
Morphology Round cells
Growth Properties Suspension, Cluster
Tissue Peripheral blood
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Catalog no. Size
302012 1 cryovial
Available Options

This cell line is available in the U.S. For non-profit users, please sign and submit the Non-Profit Supply Agreement to orders@biohippo.com before placing an order. For commercial users, please complete the CLEAR Form before ordering, as additional usage fees may apply based on the intended use. For further details, please contact orders@biohippo.com. Products ship after the required agreement is completed; typical delivery is 2–3 business days. Products are shipped frozen on dry ice in cryotubes. Each cryotube typically contains 3 × 10^6 cells for adherent lines or 5 × 10^6 cells for suspension lines (refer to the batch CoA for details).

Field Specification
Mfr No 302012
Species Human
B-LCL-CDG1 is an EBV-transformed B lymphocyte cell line derived from a patient diagnosed with PMM2-CDG, a congenital disorder of glycosylation (CDG). This rare metabolic disorder arises from mutations in the *PMM2* gene, which encodes phosphomannomutase 2 (PMM2), an essential enzyme in the glycosylation pathway. Mutations in *PMM2* disrupt the synthesis of glycosylated oligosaccharide chains, leading to defective glycosylation of various glycoproteins and glycosphingolipids in tissues and blood. The disorder is characterized by multisystemic manifestations, often affecting neurological, hepatic, and endocrine functions. As an EBV-transformed lymphoblastoid cell line, B-LCL-CDG1 provides a valuable in vitro model for studying the molecular and cellular consequences of *PMM2* deficiency. This cell line can be used to investigate glycosylation defects, PMM2 enzyme activity, and potential therapeutic interventions, including gene correction and substrate supplementation. B-LCL-CDG1, alongside other CDG patient-derived cell lines, serves as a crucial resource for understanding the pathophysiology of CDGs and evaluating novel treatment strategies for these disorders.

SKU:BHC11101674

Viruses: Transformant: EBV

  • cultureMedium: RPMI 1640, w: 2.0 mM stable Glutamine, w: 2.0 g/L NaHCO3 (Cytion article number 820700a)
  • 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.
  • fluidRenewal: Once the medium colour turned into yellow
  • freezeMedium: As a cryopreservation medium, 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.
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