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Br J Haematol ; 118(3): 858-63, 2002 Sep.
Article in English | MEDLINE | ID: mdl-12181059

ABSTRACT

Expression of common gamma chain (gammac) on monocytes was studied in five carriers of X-linked severe combined immunodeficiency (X-SCID) and two X-SCID patients who underwent cord blood stem cell transplantation (CBSCT). Flow cytometric analysis revealed that both gammac-negative and positive monocytes co-existed in X-SCID carriers, whereas no gammac-negative T, B or NK cells were observed in them. Clonal analysis and reverse transcription polymerase chain reaction studies revealed that 13.2-45.0% of monocytes from these carriers expressed the mutant gammac message. X-SCID patients who received CBSCT persistently possessed the majority of gammac-negative monocytes with a good clinical course. These results, together, may indicate that gammac is not essential for monocyte development/function in vivo.


Subject(s)
Hematopoietic Stem Cell Transplantation , Monocytes/metabolism , Receptors, Interleukin-7/genetics , Severe Combined Immunodeficiency/genetics , Female , Fetal Blood , Flow Cytometry , Genetic Linkage , Heterozygote , Humans , Interleukin Receptor Common gamma Subunit , Male , Mutation , RNA, Messenger/genetics , Receptors, Interleukin-7/blood , Reverse Transcriptase Polymerase Chain Reaction , Severe Combined Immunodeficiency/blood , X Chromosome
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