Abnormalities in DNA rearrangements of immunoglobulin gene loci in precursor B cells derived from X-linked agammaglobulinemia patient and a severe combined immunodeficiency patient
| Autor: | Ikuya Tsuge, Jun-ichi Okada, Yoshikazu Ichihara, Hiroshi Matsuoka, Hisashi Yasui, Shinpei Torii, Yoshikazu Kurosawa |
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| Rok vydání: | 1988 |
| Předmět: |
Immunoglobulin gene
Herpesvirus 4 Human Immunoglobulin delta-Chains Immunology X-linked agammaglobulinemia Germline Cell Line Agammaglobulinemia hemic and lymphatic diseases Genetics medicine Humans Gene Southern blot Recombination Genetic B-Lymphocytes Severe combined immunodeficiency biology Immunologic Deficiency Syndromes DNA Gene rearrangement Cell Transformation Viral Hematopoietic Stem Cells medicine.disease Virology Genes Immunoglobulin J-Chains biology.protein Antibody Immunoglobulin Heavy Chains |
| Zdroj: | Immunogenetics. 27:330-337 |
| ISSN: | 1432-1211 0093-7711 |
| DOI: | 10.1007/bf00395128 |
| Popis: | In an attempt to characterize the genes that cause immunodeficiencies such as X-linked agammaglobulinemia (XLA) and severe combined immunodeficiency (SCID) we established precursor B-cell lines by transforming the patients' bone marrow cells with Epstein-Barr viruses. DNA rearrangements of immunoglobulin JH gene loci were observed on both chromosomes in pre-B cells derived from an XLA patient. We cloned and characterized both rearranged bands from one cell line. Both of the rearrangements occurred between D H and J H gene loci without the VH DH structure. On the other hand, JH gene loci retained the germline configuration on both chromosomes in almost all the transformants derived from a SCID patient that had been determined according to their surface markers, to be in an early precursor B-cell stage. The implications of the observations are discussed. |
| Databáze: | OpenAIRE |
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