The role of common gamma chain in human monocytes in vivo; evaluation from the studies of X-linked severe combined immunodeficiency (X-SCID) carriers and X-SCID patients who underwent cord blood stem cell transplantation.
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.
Research Group of Human Gene Therapy, Hokkaido University Graduate School of Medicine, Teine Keijinkai Hospital, Sapporo, Japan. tada-ari@med.hokudai.ac.jp
Tadashi
T
Ariga
Koji
K
Yamaguchi
Jukei
J
Yoshida
Akihiko
A
Miyanoshita
Toshihide
T
Watanabe
Tadashi
T
Date
June-Ichi
J
Miura
Satoru
S
Kumaki
Naoto
N
Ishii
Yukio
Y
Sakiyama
D005260:Female; D005312:Fetal Blood; D005434:Flow Cytometry; D008040:Genetic Linkage; D018380:Hematopoietic Stem Cell Transplantation; D006579:Heterozygote; D006801:Humans; D053631:Interleukin Receptor Common gamma Subunit; D008297:Male; D009000:Monocytes / Q000378:metabolism*; D009154:Mutation; D012333:RNA, Messenger / Q000235:genetics; D020395:Receptors, Interleukin-7 / Q000097:blood / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016511:Severe Combined Immunodeficiency / Q000097:blood / Q000235:genetics*; D014960:X Chromosome
C507941:IL2RG protein, human; D053631:Interleukin Receptor Common gamma Subunit; D012333:RNA, Messenger; D020395:Receptors, Interleukin-7