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Biomedical subjects

N Sakai

Publications and source records attributed to N Sakai.

At least 289 records · Page 16Linked to original sources

Use of desferrioxamine in the treatment of aceruloplasminemia.

Aceruloplasminemia is a newly recognized autosomal recessive disorder of iron metabolism resulting in neurodegeneration of the retina and basal ganglia. We report here on the treatment of a patient who developed progressive extrapyramidal symptoms that included blepharospasm, grimacing, and rigidity associated with increased iron deposition in the brain and visceral organs. Treatment for 10 months with the iron chelator desferrioxamine decreased brain iron stores, prevented progression of the neurological symptoms, and reduced plasma lipid peroxidation. These data suggest that early treatment with this chelator may be useful in such patients to diminish central nervous system iron accumulation and to prevent or ameliorate neurological symptoms associated with neurodegeneration.

Ceruloplasmin↗

Primary pleomorphic adenoma in posterior cranial fossa.

A 35-year-old woman had an intradural tumor in the posterior fossa adjacent to the posterior wall of the left pyramidal bone, which was totally removed and histologically diagnosed as a pleomorphic adenoma. Follow-up examination for 2 years showed no recurrence of the tumor. There was no primary lesion in any other gland of the body, and therefore there is no alternative but to conclude a "migration" of some gland cells. The pathogenesis of this tumor remains unclassified.

Adenoma, Pleomorphic↗

Messenger RNA fingerprinting analysis using arbitrarily primed PCR (RAP) of genes expressed during rat C6 glioma cell differentiation.

To identify differentially expressed genes involved in rat C6 glioma cell differentiation induced by cyclic AMP, we adopted mRNA fingerprinting using arbitrarily primed polymerase chain reaction (PCR) (RAP). Four complementary DNA (cDNA) fragments differentially expressed during differentiation were isolated, and they appeared to contain coding regions of corresponding mRNAs. RAP can be used to efficiently identify cDNA fragments by comparing nucleotide and deduced amino acid sequences with those in databases, and is thus a useful method to search for and identify important genes involved in complex cellular processes such as glioma cell differentiation.

Animals↗

Germinoma in cerebral hemisphere associated with Down syndrome.

A Down syndrome patient with germinoma developing in the cerebral hemisphere is reported. A review of the literature yielded only 14 cases of Down syndrome with brain tumors, including our case. This finding of brain tumors in patients with Down syndrome may reflect chance occurrence. However, it is of interest in this regard that in 6 of the 14 (43%) reported cases the lesions were intracranial germ cell tumors.

Adolescent↗

Incidence of apoptotic bodies in membrana granulosa of the patients participating in an in vitro fertilization program.

OBJECTIVE: To investigate the incidence of apoptotic bodies in mural granulosa cell masses and cumulus cell masses. DESIGN: Nonrandomized, prospective study. SETTING: Department of Obstetrics and Gynecology, Yamagata University School of Medicine, Yamagata, Japan. PATIENT(S): One hundred twenty-nine normally ovulating women underwent ovulation induction for IVF-ET with GnRH analogue (GnRH-a) and gonadotropins. INTERVENTION(S): Patients underwent follicle aspiration after the administration of hCG. MAIN OUTCOME MEASURE(S): The nuclei of recovered granulosa cells were examined by fluorescence microscopy and the incidence of apoptotic bodies was tabulated. RESULT(S): The incidence of apoptotic bodies was significantly higher in mural granulosa cell masses than in cumulus cell masses in the entire group of 129 patients. Both incidence of apoptotic bodies of mural granulosa cell masses and cumulus cell masses were significantly higher in patients with less than six follicular oocytes compared with patients with six or more oocytes. Nonpregnant patients showed significantly higher incidence of apoptotic bodies in mural granulosa cell masses compared with pregnant patients. CONCLUSION(S): These results indicate that mural granulosa cell masses and cumulus cell masses may have different functions in follicular maturation. The incidence of apoptotic bodies in mural granulosa cell masses can be used as an indicator of success of IVF.

Adult↗

Increased serum remnant lipoproteins in patients with apolipoprotein E7 (apo E Suita).

Apolipoprotein (apo) E7 was originally identified by Yamamura et al. in subjects with atherosclerotic cardiovascular diseases (J. Clin. Invest. 1984;74:1229). However, the lipoprotein abnormalities associated with apo E7 phenotype have not been elucidated. In the current study, to clarify the physiological roles of apo E7, lipoprotein abnormalities were studied in 12 apo E7 heterozygotes. A total of seven subjects were hyperlipidemic and five subjects were normolipidemic. The apo E phenotype was apo E7/3 in 11 subjects and apo E7/4 in one subject. Polymerase chain reaction revealed that all of the subjects with apo E7 phenotype had the same mutation as that of apo E(Suita) as reported previously (J. Biochem. 1989;105:249). All the hyperlipidemic subjects were over 40 years of age and two of them also had and severe coronary heart disease. Ultracentrifugal analysis revealed that the cholesterol level both in very low density lipoprotein and in intermediate density lipoprotein (IDL) was substantially higher in hyperlipidemic apo E7 heterozygotes, compared with control subjects and that the IDL cholesterol was also increased even in normolipidemic apo E7 heterozygotes. Polyacrylamide gel electrophoresis of lipoproteins showed a midband, which implies the increase of remnant lipoproteins, in 11 subjects out of 12, irrespective of the presence or absence of hyperlipoproteinemia. In two cases, a broad beta pattern was observed similar to that seen in type III hyperlipoproteinemia. Dietary therapy was dramatically effective for the treatment of hyperlipidemia in patients with apo E7. These findings confirm that apo E is crucial for remnant lipoprotein metabolism and that apo E7 is related to the increase in serum remnant lipoproteins, which leads to hyperlipoproteinemia in association with obesity, aging and impaired glucose metabolism.

Adult↗

Effects of tricyclic and tetracyclic antidepressants on the three subtypes of GABA transporter.

We examined the effects of 11 antidepressants on the activities of three-rat GABA transporters (GAT1, GAT2 and GAT3) expressed in COS-7 cells. Desipramine and maprotiline inhibited all subtypes of GABA transporter, while doxepin, mianserin, trimipramine, nortriptyline and amitriptyline inhibited certain types of GABA transporters. Amitriptyline inhibited the GAT1 and GAT3 at the reasonable dosage for clinical administration. These results suggest that the inhibition of GABA uptake might have some role in the prescribed antidepressive and sedative nature of this drug.

Amitriptyline↗

Differential expression of Rho family GTP-binding proteins and protein kinase C isozymes during C6 glial cell differentiation.

The differential expression of Rho family of low molecular weight GTP-binding proteins and protein kinase C (PKC) isozymes were examined during differentiation of rat C6 glial cells to astrocytic phenotypes induced by dibutyryl cAMP (dbcAMP)/theophylline. The cells showed rapid and distinct morphological changes, resembling stellate astrocytes at 12 h after the treatment. The treated cells had a round cell body that extended several long processes each with a beaded appearance. In addition to morphological changes, Western blot analysis revealed that S-100 protein, known as a glial cell differentiation marker, increased and reached the maximal level (approximately 6-fold increase) at 24 h following the addition of dbcAMP. In the control experiments with cells cultured in the absence of serum but also without dbcAMP/theophylline, morphological changes were marginal and apparent increases of S-100 protein were not observed by Western blotting. In response to dbcAMP/theophylline treatment, RhoA showed increases in the mRNA level followed by the protein level, as inferred by reverse transcriptase-polymerase chain reaction (RT-PCR) and Western blotting, respectively. Rac1 and Cdc42 proteins were undetectable by Western blot analyses. In PKC isozymes, increases were observed in PKC beta 1, epsilon, and zeta by RT-PCR, and in beta 1 and epsilon by Western blotting. Among them, PKC epsilon showed the most distinct changes. Its mRNA level transiently increased from 3 to 6 h and then decreased even below the basal level at 18 h after the treatment. In contrast, Western blot analysis revealed that PKC epsilon gradually increased time-dependently to 24 h (approximately 6-fold increase), and remained elevated until 48 h. These results suggested that RhoA and PKC epsilon, and probably also PKC beta 1 and PKC zeta, were closely implicated in C6 cell differentiation.

Animals↗

The relationship between moyamoya disease and bacterial infection.

To examine the relationship between Moyamoya disease and bacterial infections, authors studied the serum of 85 cases of Moyamoya disease and the influence of Propionibacterium acnes (P. acnes) infection on intracranial arteries in rats. The serum levels of P. acnes antibody, IgM, transferrin (Tf), alpha 2-macroglobulin (alpha 2M) were significantly higher in Moyamoya disease than in normal volunteers. Moyamoya-like changes of the intracranial internal carotid arteries were histopathologically demonstrated in P. acnes infectious rats. These findings suggest that P. acnes and immunological factors might play a role in the pathogenesis of Moyamoya disease.

Adolescent↗

Studies on cytomegalovirus and Epstein-Barr virus infection in moyamoya disease.

In this study, measurement of Epstein-Barr virus (EBV) and cytomegalovirus (CMV) viral antibody titers and analysis of both viral genomic sequences using polymerase chain reaction (PCR) were performed to clarify the correlation of viral infection and Moyamoya disease. Serum samples were obtained from 64 patients with Moyamoya disease. The ages ranged from 5 to 66 years, with a mean age of 35.1 years. There were 23 males and 41 females. The serum antibody titers to CMV and EBV were measured by means of compliment fixation test and fluorescent antibody method respectively. Those titers of the patients were compared with those of 13 patients of atherosclerotic internal carotid occlusion and 34 normal volunteers. On the other hand, CMV and EBV genomic sequence using PCR, which was utilized with specific primer pairs, were performed in 22 patients of Moyamoya disease and ten normal volunteers. The following results were obtained; The antibody titer of EBV in Moyamoya disease was significantly higher than that in controls. However, no significant difference of antibody titer against CMV was detected. In EBV DNA analysis by use of PCR, EBV DNA was proved in 15 out of 20 patients with Moyamoya disease and four out of nine normal controls. Namely, EBV DNA was seen more frequently in patients with Moyamoya disease, compared with normal controls. In inverse, CMV DNA was not seen in patients with Moyamoya disease nor normal controls. In conclusion, the antibody titer of EBV revealed high levels in Moyamoya disease and EBV DNA was also detected more frequently in patients with Moyamoya disease. These results suggested a possibility that EBV infection might be involved in the pathogenesis of Moyamoya disease.

Adolescent↗

Simple in-office closure of small intractable tympanic membrane perforations after myringoplasty.

Perforations produced following myringoplasty are very difficult to close by ordinary cautery or patching even if they are small. Therefore we decided to close these intractable perforations with a skin graft from the cartilaginous ear canal. Our method is an in-office operation under local anesthesia. After the removal of epithelium from around the perforation a skin graft was placed by the over-lay method. A total of 11 patients were treated, of which 81.8% (9/11) demonstrated successful closure in the initial surgery and two re-perforations were successfully closed by a second and third operation, respectively. The technique used in this surgery is relatively easy and appropriate for in-office operations. The method's advantages include decreased invasiveness with minimal anesthesia and no requirement for ear canal packing or fixatives, and cosmetic benefit. This method also can be applied to perforations caused by chronic otitis media, traumatic perforations, and perforations after inserting tubes.

Adolescent↗

Clinical trial of external beam-radiotherapy combined with daily administration of low-dose cisplatin for supratentorial glioblastoma multiforme--a pilot study.

This trial tested the assumed efficacy and safety of external beam-radiotherapy combined with daily administration of low dose cisplatin (CDDP) (ERCLC therapy) for patients with glioblastoma multiforme (GBM). Thirty adult patients with supratentorial GBM received daily postoperative treatment with low dose intravenous CDDP (4-6 mg/m2) administered 30 minutes before external irradiation. In 10 patients, intraoperative radiotherapy (IORT) following surgery was given prior to ERCLC therapy. Tumor response on MRI, interval to tumor progression, survival, and toxicities were analyzed. None of the patients showed a tumor response to ERCIC therapy. Overall, the median time to tumor progression was 6 months with a 1-year tumor progression-free rate of 26.7% and a 2-year rate of 0%. The median survival time was 15 months with a 1-year survival rate of 69.9% and a 2-year rate of 31.5%. The survival rate of patients with IORT was better than that of those without IORT, however, there was no significant difference. Anorexia associated with nausea occurred in 70% and general fatigue in 10.0%. Leukopenia and thrombocytopenia occurred in 26.7% and 33.3%, respectively. However, none of the patients had to be withdrawn from therapy due to these toxicities. Other toxicities were not observed. This clinical study showed that daily administration of low dose CDDP did not enhance tumor response to irradiation for GBM on MRI. Regarding toxicity, however, ERCLC therapy was well tolerated. Although this trial did not provide sufficient data to determine whether ERCLC therapy was effective for GBM due to the small number of patients, additional clinical trials of this therapy may be warranted because that the survival rate in this study was equal to the better results recently reported for newly diagnosed GBM.

Adult↗

High-density lipoproteins from probucol-treated patients have increased capacity to promote cholesterol efflux from mouse peritoneal macrophages loaded with acetylated low-density lipoproteins.

To elucidate the anti-atherogenic effect of probucol, high-density lipoprotein (HDL) was isolated from probucol-treated patients (n = 14) and compared with that from control subjects (n = 12). The HDL obtained from probucol-treated patients was low in cholesteryl ester (CE) in comparison with that from control subjects (21.3 +/- 3.9 mol per cent vs. 27.6 +/- 3.2 mol% of total lipids. P < 0.001), and the peak diameters of patients' HDL were significantly smaller than those of control subjects on 4-30% non-denaturing polyacrylamide gradient gel electrophoresis (10.6 +/- 0.6 nm vs. 12.1 +/- 0.4 nm, P < 0.001). These data may be explained by the increased cholesteryl ester transfer protein (CETP) activities of probucol-treated patients (129 +/- 12% of control subjects, P < 0.001). The in vitro ability of HDL to remove CE from lipid-laden macrophages induced by incubation with acetylated low-density lipoprotein (Ac-LDL) was studied. The small and CE-poor HDL obtained from probucol-treated patients had a greater capacity to promote CE efflux from macrophages than did control HDL (59.8 +/- 6.9% vs. 44.2 +/- 5.4%, P < 0.01). Furthermore, the ability of HDL to promote cholesterol efflux correlated negatively with the CE content and particle diameter of HDL (r = -0.561 and r = -0.583 respectively; P < 0.01). When the inhibitory effect of HDL on the incorporation of [14C]-oleate into cellular cholesteryl ester was compared, the HDL from patients and control subjects inhibited CE formation to a similar extent. The enhanced ability of probucol-treated patients' HDL may, therefore, be involved in the acceleration of hydrolysis of the CE pool in macrophages. Taken together, we conclude that CETP plays a crucial role in making HDL more active in its anti-atherogenic function by reducing CE and making HDL smaller, and that probucol may enhance reverse cholesterol transport by activating CE transfer in vivo. The current study demonstrated, for the first time, that HDL modified by enhanced CETP activity in vivo is potentially anti-atherogenic.

Adult↗

Modulation of serotonin transporter activity by a protein kinase C activator and an inhibitor of type 1 and 2A serine/threonine phosphatases.

We studied the effects of 12-O-tetradecanoylphorbol 13-acetate (TPA), a protein kinase C (PKC) activator, and calyculin A (CLA), an inhibitor of type 1 and 2A serine/threonine phosphatases, on serotonin uptake by a human placenta choriocarcinoma cell line (BeWo) and COS-7 cells expressing recombinant serotonin transporter (SET). In BeWo cells, treatment with TPA decreased imipramine-sensitive serotonin uptake with a reduction in Vmax without affecting Km. CLA also decreased imipramine-sensitive serotonin uptake in a manner similar to that of TPA. TPA and CLA also decreased the uptake activity of recombinant SET expressed in COS-7 cells as seen in BeWo cells. These effects of TPA and CLA were reversed by staurosporine, a protein kinase inhibitor. To elucidate whether the inhibitory effects of TPA and CLA were due to direct phosphorylation of SET by PKC, site-directed mutagenesis of five putative PKC phosphorylation sites in SET was performed. Serotonin uptake was also down-regulated by TPA and CLA in all nine mutants, suggesting that these inhibitory modulation of SET activity did not act via direct phosphorylation of SET by PKC.

Adrenergic Uptake Inhibitors↗

Changes in the activity and mRNA levels of phospholipase D during ceramide-induced apoptosis in rat C6 glial cells.

N-Acetylsphingosine (C2-ceramide), a membrane-permeable analogue, induced apoptosis in C6 glial cells. Phase-contrast micrographs showed that the round cells appeared 3 h after exposure to 25 microM C2-ceramide and the number of floating cells increased time-dependently. Staining with Hoechst 33258 dye showed condensed or fragmented nuclei in round cells at 12 h. DNA fragmentation was also observed by agarose gel electrophoresis at 12 h. To understand the mechanism underlying glial cell death induced by C2-ceramide treatment, changes in phospholipase D (PLD) activity in response to guanosine 5'-O-(3-thiotriphosphate) (GTPgammaS) and expression of mRNA levels of PLD isozymes were examined. In cell lysate, GTPgammaS-dependent PLD activity was down-regulated after ceramide treatment in a time-dependent manner. In the in vitro PLD assay, membrane-associated PLD activation in response to recombinant ADP-ribosylation factor 1 was greatly suppressed. Furthermore, levels of rPLD1a and rPLD1b mRNAs were found to be down-regulated, whereas the level of rPLD2 mRNA increased gradually, peaking at 3 h, followed by a slow decrease, as inferred by reverse transcription-polymerase chain reaction. Decreases in GTPgammaS-dependent PLD activity were well correlated with those in rPLD1a and rPLD1b mRNAs levels. Taken together, these data suggest that levels of PLD enzymes might be decreased by ceramide treatment.

ADP-Ribosylation Factors↗

Purification of a factor from CSF in patient after SAH which induces the cytosolic free calcium elevation in vascular smooth muscle cells.

Cerebrospinal fluid from patients with aneurysmal subarachnoid hemorrhage induces the elevation of cytosolic free calcium [Ca2+]i in cultured vascular smooth muscle cells. We have purified a [Ca2+]i elevating factor from cerebrospinal fluid of a patient with subarachnoid hemorrhage due to aneurysm rupture. The calcium-elevating protein factor was purified to homogeneity by ammonium sulfate precipitation and a combination of Mono Q, Superose 12, and Mono S columns using liquid chromatography. Fifteen microgram of the purified protein was obtained from 340 mg of cerebrospinal fluid proteins and the molecular mass of the protein was estimated to be 81 kDa by sodium dodecyl sulfate polyacrylamide gel electrophoresis. It was also shown that the purified protein was cross-reactive with anti-human transferrin antibody. These results suggested that transferrin may be involved with the cerebral vasospasm after subarachnoid hemorrhage.

Animals↗

Molecular genetics of plasma cholesteryl ester transfer protein.

Plasma cholesteryl ester transfer protein facilitates the transfer of cholesteryl ester from HDL to apolipoprotein B-containing lipoproteins, and is a key protein in the reverse cholesterol transport system. The importance of plasma cholesteryl ester transfer protein in lipoprotein metabolism was highlighted by the discovery of deficient individuals with a marked hyper-HDL-cholesterolemia. Cholesteryl ester transfer protein deficiency causes various abnormalities in the concentration, composition, and functions of both HDL and LDL. Its significance in atherosclerosis is still controversial. However, in-vitro evidence shows large cholesteryl ester-rich HDL particles in cholesteryl ester transfer protein deficiency are defective in cholesterol efflux. Recent epidemiological studies have demonstrated an increased incidence of coronary atherosclerosis in deficient patients. The current review will also focus on the molecular genetics of the protein.

Animals↗

Aldosterone-producing adrenocortical carcinoma metastases found seven years after adrenalectomy.

We report a case of metastatic adrenocortical carcinoma detected 7 years after adrenalectomy. A 52-year-old woman, who had undergone adrenalectomy for an aldosterone-producing adrenocortical carcinoma at age 45, was found on examination by computerized tomography to have enlarged paraaortic lymph nodes. These nodes were surgically resected, and the histological diagnosis from the resected tissue was metastatic adrenocortical carcinoma. The patient has now survived for more than 9 years following the original adrenalectomy. Evidence suggests that this was a slow-growing tumor, because the primary tumor was sharply demarcated and the metastases were found 7 years after the original operation. We believe that aggressive surgical resection of metastatic lesions could lead to prolonged survival in patients with adrenocortical carcinomas of this type.

Adrenal Cortex Neoplasms↗