LB199

DNA repair deficiency studies

Human lymphocyte, trichothiodystrophy, EBV-transformed

LB199 was derived from mononuclear cells from a peripheral blood sample from a 16 year old male. The patient was diagnosed with trichothiodystrophy and was shown to have a severe deficiency in excision repair of UV-irradiation damage. The defect was able to complement all known Xeroderma pigmentosum complementation groups and was not intragenic. This cell line is therefore a representative of a new excision-repair complementation group. The cell line was established by subsequent EBV transformation. Fibroblasts from the same individual have been designated TTD1BR. EBV-transformed lymphoblastoid cells from the mother are available as LB198 (Sigma Catalogue number. 98062320).

Maintain cultures between 3-9 x 100,000 cells/ml; dilute every 1 -2 days to 3 x 100,000 cells/ml; 5% CO₂; 37°C. Cells form floating aggregates.

RPMI 1640 + 4mM Glutamine + 200 μg/ml Sodium Pyruvate (NaPy) + 15% Heat Inactivated (HI-FBS).

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This cell line is part of the European Collection of Authenticated Cell Cultures (ECACC), an international repository managed by the United Kingdom Health Security Agency (UKHSA). No licensing agreement is required when either this cell line or the DNA extracted from it are used for internal research purposes only. Any other use of these products is prohibited without the express written permission of UKHSA. Inquiries regarding authorized use of this cell line or its genomic DNA may be directed to culturecollections@ukhsa.gov.uk.