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.
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Authors | Tadashi Ariga, Koji Yamaguchi, Jukei Yoshida, Akihiko Miyanoshita, Toshihide Watanabe, Tadashi Date, June-Ichi Miura, Satoru Kumaki, Naoto Ishii, Yukio Sakiyama |
Journal | British journal of haematology
(Br J Haematol)
Vol. 118
Issue 3
Pg. 858-63
(Sep 2002)
ISSN: 0007-1048 [Print] England |
PMID | 12181059
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- IL2RG protein, human
- Interleukin Receptor Common gamma Subunit
- RNA, Messenger
- Receptors, Interleukin-7
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Topics |
- Female
- Fetal Blood
- Flow Cytometry
- Genetic Linkage
- Hematopoietic Stem Cell Transplantation
- Heterozygote
- Humans
- Interleukin Receptor Common gamma Subunit
- Male
- Monocytes
(metabolism)
- Mutation
- RNA, Messenger
(genetics)
- Receptors, Interleukin-7
(blood, genetics)
- Reverse Transcriptase Polymerase Chain Reaction
- Severe Combined Immunodeficiency
(blood, genetics)
- X Chromosome
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