IMMUNE REGULATION
Identification of FOXP3-negative regulatory T-like
(CD4
+
CD25
+
CD127
low
) cells in patients with
immune dysregulation, polyendocrinopathy,
enteropathy, X-linked syndrome
Keisuke Otsubo
a
, Hirokazu Kanegane
a ,
⁎
, Yoshiro Kamachi
d
,
Ichiro Kobayashi
e
, Ikuya Tsuge
f
, Masue Imaizumi
g
, Yoji Sasahara
h
,
Akira Hayakawa
i
, Kandai Nozu
i
, Kazumoto Iijima
i
, Shuichi Ito
j
,
Reiko Horikawa
k
, Yoshinori Nagai
b
, Kiyoshi Takatsu
b , l
, Hisashi Mori
c
,
Hans D. Ochs
m
, Toshio Miyawaki
a
a
Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan
b
Department of Immunobiology and Pharmacological Genetics, Graduate School of Medicine and Pharmaceutical Sciences,
University of Toyama, Toyama, Japan
c
Department of Molecular Neuroscience, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama,
Toyama, Japan
d
Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
e
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
f
Department of Pediatrics, Fujita Health University School of Medicine, Toyoake, Japan
g
Department of Hematology and Oncology, Miyagi Children's Hospital, Sendai, Japan
h
Department of Pediatrics, Tohoku University Graduate School of Medicine, Sendai, Japan
i
Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
j
Department of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan
k
Department of Endocrinology, National Center for Child Health and Development, Tokyo, Japan
l
Toyama Prefectural Institute for Pharmaceutical Research, Toyama, Japan
m
Department of Pediatrics, University of Washington School of Medicine and Seattle Children's Research Institute, Seattle, USA
Received 10 November 2010; accepted with revision 22 June 2011
Available online 12 July 2011
Abbreviations APC, antigen-presenting cells; FKH, forkhead; FOXP3, forkhead box protein 3; HSCT, hematopoietic stem cell
transplantation; mAb, monoclonal antibodies; MLR, mixed lymphocyte reaction; IPEX, immune dysregulation, polyendocrinopathy,
enteropathy, X-linked; PBMC, peripheral blood mononuclear cell; Treg, regulatory T
⁎ Corresponding author at: Department of Pediatrics, Graduate School of Medicine, University of Toyama, 2630 Sugitani, Toyama, Toyama
930–0194, Japan. Fax: +81 76 434 5029.
E-mail address: kanegane@med.u-toyama.ac.jp (H. Kanegane).
1521-6616/$ - see front matter © 2011 Elsevier Inc. All rights reserved.
doi:10.1016/j.clim.2011.06.006
Clinical Immunology (2011) 141, 111–120
available at www.sciencedirect.com
Clinical Immunology
www.elsevier.com/locate/yclim