721 Wang, et al: PD-L gene in SLE
Ligands for Programmed Cell Death 1 Gene in Patients
with Systemic Lupus Erythematosus
SHU-CHEN WANG, CHIA-HUI LIN, TSAN-TENG OU, CHENG-CHIN WU, WEN-CHAN TSAI, CHAUR-JONG HU,
HONG-WEN LIU, and JENG-HSIEN YEN
ABSTRACT. Objective. To investigate the role of ligands for programmed cell death 1 (PD-L) in the pathogenesis of
systemic lupus erythematosus (SLE).
Methods. One hundred sixty-four patients with SLE and 160 healthy controls were enrolled in our
study. The PD-L1 and PD-L2 polymorphisms were determined by polymerase chain reaction
(PCR)/direct sequencing or restriction fragment length polymorphism (RFLP)-PCR.
Results. The genotype distributions of PD-L2 47103 C/T polymorphisms in patients with SLE were sig-
nificantly different from those of the controls (p = 0.003). The genotype frequency of PD-L2 47103 T/T,
in comparison with 47103 C/C, was significantly increased in patients with SLE when compared with
that of the controls (odds ratio 2.5, 95% confidence interval 1.4–4.4, p = 0.001). A similar finding could
also be found in the allele frequency of PD-L2 47103 T (SLE vs control, OR 1.7, 95% CI 1.3–2.4,
p = 0.001). There were no significant differences in the genotype and allele frequencies of PD-L1 poly-
morphisms between the patients and controls.
Conclusion. PD-L2 47103 T may be associated with susceptibility to SLE in Taiwan. (First Release
Mar 1 2007; J Rheumatol 2007;34:721–5)
Key Indexing Terms:
PROGRAMMED CELL DEATH 1 LIGAND PROGRAMMED CELL DEATH 2 LIGAND
SYSTEMIC LUPUS ERYTHEMATOSUS PROGRAMMED CELL DEATH 1
From the Department of Laboratory Medicine and Division of
Rheumatology, Department of Internal Medicine, Kaohsiung Medical
University Hospital; College of Medicine and Graduate Institute of
Medicine, College of Medicine, Kaohsiung Medical University,
Kaohsiung; and the Department of Neurology, Taipei Medical University
Hospital, Taipei, Taiwan.
S-C. Wang, MSc, Department of Laboratory Medicine; C-H. Lin, MSc;
T-T. Ou, MD, Attending Physician; C-C. Wu, MD, Attending Physician,
Division of Rheumatology, Department of Internal Medicine, Kaohsiung
Medical University Hospital; W-C. Tsai, MD, PhD, Associate Professor;
H-W. Liu, MD, Professor, Division of Rheumatology, Department of
Internal Medicine and College of Medicine, Kaohsiung Medical
University; C-J. Hu, MD, Assistant Professor, Department of Neurology,
Taipei Medical University Hospital; J-H. Yen, MD, PhD, MSc, Professor,
Division of Rheumatology, Department of Internal Medicine and
Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical
University.
Address reprint requests to Dr. J-H. Yen, Division of Rheumatology,
Department of Internal Medicine, Kaohsiung Medical University
Hospital, No. 100 Zih-You 1st Road, Kaohsiung City 807, Taiwan.
E-mail: jehsye@kmu.edu.tw
Accepted for publication December 28, 2006.
Systemic lupus erythematosus (SLE) is a prototype of autoim-
mune diseases, which frequently involves multiple organs.
The detailed pathogenesis is still obscure. Many genes such as
human leukocyte antigen, complement, FcrR, mannose bind-
ing lectin, protein tyrosine phosphatase N22, cytokines, and
chemokine genes have been described to be associated with
the development of SLE
1-5
.
Programmed cell death 1 (PD-1) is an immunoinhibitory
receptor expressed by activated T cells, B cells, and myeloid
cells
6
. The ligands for PD-1 (PD-L1 and PD-L2, also known
as B7-H1 and B7-DC) are type I transmembrane proteins
structurally related to the B7 family. They can be induced in
monocytes, dendritic cells, endothelial cells, keratinocytes,
and B cells
6-10
. However, PD-L1 expression is different from
that of PD-L2. PD-L1 is also expressed on activated T cells,
placental trophoblasts, myocardial endothelium, and cortical
thymic epithelial cells. In contrast, PD-L2 can also be pre-
sented on placental endothelium and medullary thymic epithe-
lial cells
10
. The interactions of PD-1 with PD-L1 and PD-L2
result in the inhibition of T cell receptor-mediated lymphocyte
proliferation and cytokine secretion, and they also inhibit
CD28-mediated costimulation. The relative levels of inhibito-
ry PD-L1 and costimulatory CD80/CD86 signals on antigen-
presenting cells determine the extent of T cell activation and
the threshold between tolerance and autoimmunity. Therefore,
PD-L1 expression on nonlymphoid tissues and its interaction
with PD-1 may determine the extent of immune responses at
sites of inflammation
6
.
Recent studies using anti-PD-L1 monoclonal antibodies
have suggested a role for PD-L1 in regulating autoimmune
diseases. Blockade of the PD-L during experimental autoim-
mune encephalomyelitis (EAE) or diabetes exacerbates the
diseases
11,12
. PD-L1 blockade rapidly precipitated diabetes in
prediabetic female non-obese diabetic (NOD) mice. PD-L2
blockade in animals also resulted in augmentation of EAE.
These studies showed that PD-1–PD-L blockade was related
to the development of autoimmune disease.
The polymorphisms in exons of PD-L1 and PD-L2 may
result in amino acid substitution, structural changes, and
expression of PD-L1 and PD-L2. The consequent interactions
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