ABSTRACT
Capicua (CIC) is a transcriptional repressor that regulates several developmental processes. CIC deficiency results in lymphoproliferative autoimmunity accompanied by expansion of CD44hiCD62Llo effector/memory and follicular Th cell populations. Deletion of Cic alleles in hematopoietic stem cells (Vav1-Cre-mediated knockout of Cic) causes more severe autoimmunity than that caused by the knockout of Cic in CD4+CD8+ double positive thymocytes (Cd4-Cre-mediated knockout of Cic). In this study, we compared splenic CD4+ T cell activation and proliferation between whole immune cell-specific Cic-null (Cicf/f;Vav1-Cre) and T cell-specific Cic-null (Cicf/f;Cd4-Cre) mice. Hyperactivation and hyperproliferation of CD4+ T cells were more apparent in Cicf/f;Vav1-Cre mice than in Cicf/f;Cd4-Cre mice. Cicf/f;Vav1-Cre CD4+ T cells more rapidly proliferated and secreted larger amounts of IL-2 upon TCR stimulation than did Cicf/f;Cd4-Cre CD4+ T cells, while the TCR stimulation-induced activation of the TCR signaling cascade and calcium flux were comparable between them. Mixed wild-type and Cicf/f;Vav1-Cre bone marrow chimeras also exhibited more apparent hyperactivation and hyperproliferation of Cic-deficient CD4+ T cells than did mixed wild-type and Cicf/f;Cd4-Cre bone marrow chimeras. Taken together, our data demonstrate that CIC deficiency at the beginning of T cell development endows peripheral CD4+ T cells with enhanced T cell activation and proliferative capability.
ABSTRACT
Capicua (CIC) is a transcriptional repressor that regulates several developmental processes. CIC deficiency results in lymphoproliferative autoimmunity accompanied by expansion of CD44hiCD62Llo effector/memory and follicular Th cell populations. Deletion of Cic alleles in hematopoietic stem cells (Vav1-Cre-mediated knockout of Cic) causes more severe autoimmunity than that caused by the knockout of Cic in CD4+CD8+ double positive thymocytes (Cd4-Cre-mediated knockout of Cic). In this study, we compared splenic CD4+ T cell activation and proliferation between whole immune cell-specific Cic-null (Cicf/f;Vav1-Cre) and T cell-specific Cic-null (Cicf/f;Cd4-Cre) mice. Hyperactivation and hyperproliferation of CD4+ T cells were more apparent in Cicf/f;Vav1-Cre mice than in Cicf/f;Cd4-Cre mice. Cicf/f;Vav1-Cre CD4+ T cells more rapidly proliferated and secreted larger amounts of IL-2 upon TCR stimulation than did Cicf/f;Cd4-Cre CD4+ T cells, while the TCR stimulation-induced activation of the TCR signaling cascade and calcium flux were comparable between them. Mixed wild-type and Cicf/f;Vav1-Cre bone marrow chimeras also exhibited more apparent hyperactivation and hyperproliferation of Cic-deficient CD4+ T cells than did mixed wild-type and Cicf/f;Cd4-Cre bone marrow chimeras. Taken together, our data demonstrate that CIC deficiency at the beginning of T cell development endows peripheral CD4+ T cells with enhanced T cell activation and proliferative capability.
ABSTRACT
A gastric carcinoma with the endoscopic features resembling a submucosal tumor (SMT) is rare, and reportedly accounting for 0.1% to 0.63% of all resected gastric carcinomas in Japan. A diagnosis of a SMT-like gastric carcinoma is often difficult as the tumors are almost entirely covered with normal mucosa. Furthermore mucinous gastric adenocarcinoma is uncommon histologic subtype of gastric cancer. These tumors are detected mostly in an advanced stage and rarely in an early stage. Early mucinous gastric adenocarcinoma is characterized as an elevated lesion resembling SMT due to abundant mucin pools in the submucosa. Here we report one case of SMT-like mucinous gastric adenocarcinoma, diagnosed by the usual endoscopic biopsy and treated with surgery.
Subject(s)
Aged , Humans , Male , Adenocarcinoma, Mucinous/diagnosis , Diagnosis, Differential , Gastric Mucosa/pathology , Gastroscopy , Stomach Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
Pseudohypoparathyroidism is a rare disease that is characterized by target cell resistance to the effects of parathyroid hormone and this disease is classified into various types depending on the phenotypic and biochemical findings. The patients with pseudohypoparathyroidism present with the clinical and biochemical features of hypoparathyroidism, but they have an increased serum level of parathyroid hormone. We experienced a case of pseudohypoparathyroidism in a 24 years old woman who had Graves' disease at that time. She had hypocalcemia, hyperphosphatemia, an elevated serum parathyroid hormone level and a normal urinary basal cyclic AMP(adenosine monophosphate) level. She also had a normal phenotypic appearance. Therefore, she was classified as suffering with pseudohypoparathyroidism type II. The clinical and laboratory abnormalities were improved by calcium supplementation in addition to vitamin D. To the best of our knowledge, this is the first case of pseudohypoparathyroidism combined with Graves' disease in Korea.
Subject(s)
Female , Humans , Calcium , Graves Disease , Hyperphosphatemia , Hyperthyroidism , Hypocalcemia , Hypoparathyroidism , Korea , Parathyroid Hormone , Pseudohypoparathyroidism , Rare Diseases , Stress, Psychological , Vitamin DABSTRACT
Intestinal intussusception in adults is a rare disease. Most of the cases of adult intussusception are secondary to a definable lesion, and so surgical treatment generally needed. Intussusception that occurs after colonoscopic polypectomy is apparently a rare malady. A 77-year old man undergoing colonoscopic polypectomy was diagnosed as having colonic intussusception at the hepatic flexure. As there was no clinical improvement with conservative treatment, he underwent segmental resection of the ascending and transverse colon. Pathologic examination revealed that the colonoscopic polypectomy site was a leading point of the intussusception.
Subject(s)
Adult , Humans , Colon , Colon, Transverse , Colonoscopy , Intussusception , Rare DiseasesABSTRACT
No abstract available.