PubMed Health⌕ Search

Biomedical subjects

T Okadome

Publications and source records attributed to T Okadome.

At least 19 recordsLinked to original sources
PubMed1995

Cloning and characterization of a human type II receptor for bone morphogenetic proteins.

Bone morphogenetic proteins (BMPs) are members of the transforming growth factor beta superfamily. Several members of this family have been shown to transduce their signals through binding to type I and type II serine-(threonine) kinase receptors. Here we report the cDNA cloning and characterization of a human type II receptor for BMPs (BMPR-II), which is distantly related to DAF-4, a BMP type II receptor from Caenorhabditis elegans. In transfected COS-1 cells, osteogenic protein (OP)-1/BMP-7, and less efficiently BMP-4, bound to BMPR-II. BMPR-II bound ligands only weakly alone, but the binding was facilitated by the presence of previously identified type I receptors for BMPs. Binding of OP-1/BMP-7 to BMPR-II was also observed in nontransfected cell lines. Moreover, a transcriptional activation signal was transduced by BMPR-II in the presence of type I receptors after stimulation by OP-1/BMP-7.

Amino Acid Sequence↗
PubMed1995

A rat pituitary tumor cell line (GH3) expresses type I and type II receptors and other cell surface binding protein(s) for transforming growth factor-beta.

A rat pituitary tumor cell line (GH3) has been reported to express transforming growth factor-beta (TGF-beta) binding components of 70-74 kDa (ligand included), denoted TGF-beta type IV receptor. We investigated whether the type IV receptor corresponds to any of the recently cloned type I receptors for proteins in the TGF-beta super-family. TGF-beta type I receptor (T beta R-I) complexes of 69-72 kDa formed a heteromeric complex with T beta R-II in GH3 cells, as detected by immunoprecipitation. In addition, TGF-beta formed complexes of 72-74 kDa, which were different from T beta R-I and the other known type I receptors, and were not dependent on T beta R-II for binding. The GH3 cells were resistant to the growth inhibitory activity of TGF-beta, but a transcriptional response was activated by TGF-beta in this cell line, presumably through the T beta R-II and T beta R-I complex. These results indicate that GH3 cells have T beta R-I and T beta R-II and, in addition, other binding protein(s) which form 72-74-kDa complexes with TGF-beta; the function of the latter component(s) remains to be elucidated.

Activins↗
PubMed1992

Urinary excretion of parathyroid hormone-related protein as a predictor of hypercalcemia in patients with adult T-cell leukemia.

Hypercalcemia with adult T-cell leukemia (ATL) is chiefly caused by an excessive production by tumor cells of parathyroid hormone-related protein (PTHrP). We have previously reported hypercalcemic patients with solid tumors to excrete a large amount of the C-terminal fragments of PTHrP (C-PTHrP) into their urine. To elucidate whether PTHrP production correlates with or predicts the development of hypercalcemia, we studied the urinary excretion of C-PTHrP in 36 ATL patients. The urinary excretion of C-PTHrP was in the normal range (< 0.40 nmol equivalent to PTHrP (109-141)/g creatinine) in HTLV-1-positive carriers (n 3), ATL patients in complete remission (n 2) and chronic type ATL patients (n 2). It was marginally increased in seven patients in partial remission, and gradually increased as the disease progressed. In 20 patients who died without or with hypercalcemia, it was increased to 1.98 +/- 0.69 (n 9) and 7.6 +/- 2.1 nmol/g creatinine (mean +/- SD, n 11, P < 0.01), respectively. Urinary C-PTHrP excretion was significantly correlated with serum calcium and LDH levels as well as with CD25-positive cells in the peripheral blood. In four patients whose urinary excretion had been serially determined, it increased prior to the development of hypercalcemia. The findings suggest the urinary excretion of C-PTHrP to be of use as a predictor of the development of hypercalcemia in ATL patients. In ATL patients whose urinary excretion of C-PTHrP is progressively increasing, the serum calcium concentration should be carefully monitored to prevent hypercalcemic crisis.

Adult↗
PubMed1991

Immunocytochemical study on endothelial markers with the cerebral vessel from a patient with familial moyamoya disease.

The immunocytochemical study of thrombomodulin (TM), a newly recognized anticoagulant endothelial surface protein, was performed with a surgical specimen of a superficial temporal artery (STA) obtained from a 29-year-old woman with familial moyamoya disease. The staining of TM showed positive immunoreactivity in smaller vessels in the surrounding connective tissue of the specimen, whereas negative in STA. Immunoelectronmicroscopically the luminal plasma membrane of endothelial cells was positive for TM. These staining pattern was the same as that in controls. She concurrently suffered from von Willebrand disease type I, and she had two cerebral hemorrhagic attacks. A quantitative defect of the von Willebrand factor in the endothelium was demonstrated immunocytochemically.

Adult↗
PubMed1991

[A case of scapuloperoneal atrophy with rigid spine having lobulated fibers in muscle biopsy].

The patient, a 52-year-old male, noticed abnormalities on walking at about 20 years of age, followed by slowly progressive muscle weakness of arms and neck. The family history was negative. He showed muscular atrophy and weakness with a preferential involvement of the scapular, arms and peroneal muscles. Deep tendon reflexes were absent. He had a limited range of motion in the spine, but the onset was unclear. Creatine kinase (CK) was elevated (324 IU/L) and the EMG study showed myogenic pattern. Muscle biopsy was obtained from the biceps brachii muscle; on NADH dehydrogenase stain, there was subsarcolemmal increase in the oxidative enzyme activity showing "lobulated fiber" mostly seen in type 1 fibers. On electron-microscopy, the sub-sarcoplasmic areas which had high NADH activity, contained many mitochondria and glycogen particles. However, iodine-glycogen complex spectrum analysis pattern and debranching enzyme activity were normal. CT scan revealed low density in the paravertebral muscles, suggesting degeneration. This is a rare type of scapuloperoneal atrophy different from Emery-Dreifuss syndrome, rigid spine syndrome and FSH type muscular dystrophy.

Adult↗
PubMed1991

[A study on the frequency of von Willebrand factor deficiency state].

In order to elucidate the prevalence of von Willebrand factor (vWF) deficiency state, we investigated plasma vWF levels among 343 patients with bleeding tendency, 465 patients without bleeding tendency and 704 random unrelated normals living in Kagoshima prefecture. The prevalence of symptomatic vWF deficiency state was at least 2.3 per 100,000 of the population, and that of asymptomatic one in healthy individuals was 1.3%. These results indicate that the overall incidence of vWF deficient state in general population would be considerably higher than that previously reported.

Adolescent↗
PubMed1990

[Clinical characteristics of anti-phospholipid antibodies].

Twenty-one patients with anti-phospholipid antibodies (APLA), who were selected from 104 patients with antinuclear antibody or anti-DNA antibody, were studied to define clinical characteristics of APLA. Of the 21 patients, the incidences of IgG anti-cardiolipin antibody (ACA), IgM ACA, lupus anticoagulant (LAC) and BFP-STS were 20, 7, 11 and 12, respectively, and they were highly related with each other. The number of items of the 1982 ARA revised criteria for the classification of SLE was significantly low (mean value = 3.3), and also the level of serum C 4 was significantly high (mean value = 88% of normal) compared with those in patients without APLA. The incidence of thrombocytopenia and hemolysis was significantly high when compared with those in patients without APLA, and they were closely related to the presence of LAC. The incidence of thrombosis was markedly high (48%), 10 of 21 patients, especially those of cerebral infarction and deep vein thrombosis. Cerebral infarction was significantly associated with LAC. The obstetric complication was 4 of 13 patients (31%) in the incidence, which was significantly high compared with that in patients without APLA. Of them, natural abortion was closely associated with LAC. Based on these observations, it seems that autoimmunological disease activity in patients with APLA may be slight to mild, and strongly suggested that APLA may play an important role particularly in the pathogenesis of acquired thrombotic tendency.

Adolescent↗
PubMed1990

[Pure red cell aplasia with monoclonal gammopathy and von Willebrand disease].

We report a case of pure red cell aplasia (PRCA) with benign monoclonal gammopathy (BMG) of IgA.lambda type and type I von Willebrand disease (vWD). A 61-year-old female patient was treated initially with prednisolone, azathioprine and cyclophosphamide with transient and unsatisfactory reticulocyte response. Oral administration of 200 mg of cyclosporine A (CyA)/day was started from July, 1987. A rapid and marked reticulocytosis was seen from a week later and there was a rapid increase in hemoglobin levels, and remission has been maintained for over 22 months. Patient's serum and IgA taken on admission did not show inhibitory activity to both CFU-E growth from her own bone marrow cells obtained in remission and von Willebrand factor. T cell-mediated suppression to CFU-E growth was detected. On family study, the patient's second son was found to be a type I vWD. These results indicate that there is no direct causal relationships between BMG and PRCA or vWD, and that CyA may have a place in the management of PRCA.

Cyclosporins↗
PubMed1990

[Collagen adhesion-aggregation abnormality (2nd report)--congenital platelet membrane glycoprotein VI deficiency].

Previously, we reported a case of 26-year-old woman with a mild bleeding tendency whose platelets specifically lacked collagen-induced aggregation and adhesion to collagen fibrils. In this report, we investigated the membrane glycoproteins of this patient's platelets and found that her platelets were absent in a 61-kDa glycoprotein, which was identified to be glycoprotein VI (GP VI) of the platelet membrane. Her parents platelets contained about 50% the normal amount of GP VI. These results indicate that our patient has a congenital homozygous GP VI deficiency and that GP VI functions as a collagen receptor.

Adult↗
PubMed1990

[Fetal survival after the corticosteroid and low-dose aspirin regimen in a case of anti-phospholipid antibody syndrome].

A 27-year-old woman with anti-phospholipid antibody syndrome, whose first pregnancy had ended in intrauterine fetal death, was treated with prednisolone 0.6 mg/kg/day before second pregnancy. Suppression of anti-cardiolipin antibody activity was rapidly achieved and maintained during the pregnancy. After conception, low-dose aspirin 81 mg/day was started and continued until delivery. She gave birth to a normal live infant with natural full-term delivery. It is strongly suggested that the corticosteroid and low-dose aspirin regimen can lead to successful pregnancy in cases with anti-phospholipid antibody syndrome like the present case.

Adult↗
PubMed1990

[A family with von Willebrand disease and hypofibrinogenemia].

A family with two complex disorders of hemostasis, von Willebrand disease (vWD) and hypofibrinogenemia was reported. The probands were 21- and 16-year-old full brothers suffering from serious bleeding tendencies from childhood. The elder brother had a subarachnoid hemorrhage at the age of 15. The younger brother had repeated episodes of gastrointestinal bleedings since he was 10 years of age. Coagulation studies revealed that both of them had almost the same hemostatic abnormalities, i.e. severe vWD, 9 to 12% of plasma vWF levels, and mild hypofibrinogenemia, 125 to 130 mg/dl of plasma fibrinogen levels. Multimeric compositions of their vWF were normal, and functional assay for fibrinogen concentration yielded essentially the same values as did immunologic assay. These results indicated that they had two complex disorders, extreme type I vWD and heterozygous state of afibrinogenemia resulting in serious bleeding tendency. Family study showed that these two hemostatic disorders were paternal inheritance, and it was strongly postulated that vWD and hypofibrinogenemia might be highly-combined hemostatic disorders.

Adolescent↗
PubMed1990

[T-cell malignant lymphoma with hemophagocytic histiocytosis, hyperferritinemia and disseminated intravascular coagulation syndrome].

A 57-year-old man was admitted to our hospital with high fever and nasal obstruction. The diagnosis of T cell type malignant lymphoma (T-ML) was made by the biopsy of left nasal cavity tumor. After admission, his general condition was improved by chemotherapy and radiotherapy, but relapsed a month later. He was then treated with chemotherapy, and the partial remission was obtained. During the clinical course, he had a high fever again without any significant infections or exacerbation of T-ML. The data of coagulation system showed DIC. The levels of serum ferritin and LDH were extremely elevated. Bone marrow aspiration showed markedly increased hemophagocytic histiocytes. These data suggested that he was complicated by DIC and hyperferritinemia closely associated with hemophagocytic histiocytosis a part from the underlying T-ML. Causes of DIC and hyperferritinemia associated with hemophagocytic histiocytosis in the present case were discussed.

Disseminated Intravascular Coagulation↗
PubMed1990

[A case of the primary mediastinal seminoma invading into the extradural space of the thoracic spine].

Seminomatous germ cell tumors can arise as primary mediastinal malignancies in the mediastinum without involving the testis. The usual location is the superior mediastinum and histologically it is identical to the testicular seminoma. Here we report a case of extradural spinal cord tumor by primary mediastinal seminoma. The patient, 24 year old male, was evaluated for complaints of severe back pain associated with dysesthesia in lower limbs. He was operated as primary mediastinal seminoma when he was 21 years old. On admission, physical examination was unremarkable except for hypereflexia in lower extremities. The testis were normal. Chest radiography and computed tomography revealed a large mediastinal mass which invaded into the extradural space of the thoracic spine. He had a laminectomy of the thoracic spine and was treated by the radiation therapy and chemotherapy. This is the first report showing the neuroradiological findings of the extradural spinal cord tumor by primary mediastinal seminoma.

Adult↗
PubMed1990

[Coincidental occurrence of idiopathic thrombocytopenic purpura in a patient with familial von Willebrand disease].

A very rare case with coincidental idiopathic thrombocytopenic purpura (ITP) and familial von Willebrand disease (vWD) was reported. A 23-year-old female was admitted because of unusual bleeding tendency lasting still after the remission of thrombocytopenic state associated with ITP. Coagulation studies indicated the presence of a mild vWD, which was thought to be responsible for the bleeding tendency. The qualitative analysis of von Willebrand factor by crossed immunoelectrophoresis and SDS-1.2% agarose gel electrophoresis showed normal multimeric composition compatible with type I vWD. vWF-inhibitor was negative, and family study revealed that her mother was also affected with type I vWD. These results suggested that there was no immediate causal relation between the two disease states. The prevalence of concomitant disease states with ITP and vWD was discussed.

Adult↗
PubMed1990

[Occurrence of hemophilia A and von Willebrand disease in the same family].

Occurrence of the two congenital hemorrhagic disorders, von Willebrand disease (vWD) and hemophilia A, was found in the same family. The propositus was a 21-year-old male patient with findings of moderate hemophilia A. Father, the eldest sister and second eldest sister were found to have mild type I vWD. Mother was confirmed to be a hemophilia A carrier, and the eldest son of second eldest sister to be a moderate hemophilia A. These results suggest that the occurrence of the two hemorrhagic disorders may have been resulted from the incidental mating of father's vWD gene and mother's hemophilia A gene, and suggest that second eldest sister may be the double heterozygosity of type I vWD and hemophilia A.

Adult↗
PubMed1990

[Occurrence of subdural hematoma closely associated with danazol administration in a patient with refractory ITP].

We reported a 34-year-old female patient with refractory idiopathic thrombocytopenic purpura (ITP) in whom a subacute subdural hematoma occurred without any preceding trauma during danazol administration which resulted in a marked decrease of plasma fibrinogen level. It is strongly suggested that danazol should be very carefully administered in ITP patients with serious bleeding tendency.

Adult↗