A new family of homeobox genes encoding multiple homeodomain and zinc finger motifs.
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Animals↗
Biomedical subjects
T Hashimoto
Publications and source records attributed to T Hashimoto.
Functional expression of cDNAs for bovine 11 beta-hydroxylase-aldosterone synthases, P450(11 beta)-2 and -3 and their chimeras.
Two molecular species of bovine P450(11 beta), P450(11 beta)-2 and P450(11 beta)-3 have been identified, in which the amino acid differences were found at the 6th, 36th and 82nd positions from the NH2-termini of the mature proteins. They catalyzed the 11 beta-, 18- and 19-hydroxylation and aldosterone formation from 11-deoxycorticosterone, and the rate of production of 18-hydroxycorticosterone and aldosterone by P450(11 beta)-3 was greater than that by P450(11 beta)-2 [Morohashi et al., J. Biochem. 107 (1990) 635-640]. In this study, chimeric clones were constructed whose 6th, 36th and 82nd amino acid residues were exchanged with each other. Two original clones and six chimeric clones were expressed in COS-7 cells, and their steroidogenic activities studied. The ratio of aldosterone or 18-hydroxycorticosterone production to corticosterone production by one clone was compared with that of the other. The ratios for the four clones having Gly36 [P450(11 beta)-3 type] were 0.08-0.22, whereas those for the clones having Ser36 [P450(11 beta)-2 type] were 0.03-0.05, suggesting that the Gly36 structure is important for aldosterone production.
Animals↗
Reduced brainstem size in children with autism.
Recently, structural brain abnormalities as well as functional abnormalities of the brainstem have been reported in autistic children. The authors undertook an analytic study of the brainstem in autistic children by means of magnetic resonance imaging (MRI). The MRI scans of 29 autistic children were compared with 15 control MRI scans. The autistic children were divided into two groups according to DQ (IQ) level: the DQ (IQ) greater than or equal to 80 group and the DQ (IQ) less than 80 group. The midbrain and pons were measured, and the ratio of the midbrain and pons sizes versus the cranium size were calculated. The brainstem size was found to be significantly smaller in the autistic group. In particular, the reduction in brainstem size tended to be greater in the low DQ (IQ) group when compared with the high DQ (IQ) one, though there was no significant difference (p less than 0.1). This suggests that the brainstem is anatomically altered in autistic children.
Autistic Disorder↗
Occipital deep white matter hyperintensity as seen by MRI: 1. Clinical significance.
Magnetic resonance imaging was performed in 270 patients with various neurologic complaints (1-15Y) with a 0.5 tesla superconducting imaging system using a field echo T1-weighted sequence and spin echo T2-weighted and PD-weighted sequences. Twenty-seven of them had deep white matter hyperintensity (DWMH) in the occipital lobe on T2-weighted images. The frequency of mild DWMH differed in different age groups, suggesting that mild DWMH may result from delayed myelination in the central nervous system. However, the frequency of severe DWMH, which was revealed as isointense relative to cerebrospinal fluid, did not differ in different age groups and it was significantly more common in severely retarded patients. Classification of DWMH based on the signal intensity is valuable to distinguish white matter abnormalities in the occipital lobe from delayed myelination in the same site.
Adolescent↗
Electrophysiological study on hydranencephaly.
An electrophysiological study was performed on 2 children with hydranencephaly diagnosed by CT and/or MRI. Case 1 was a 4-month-old boy who had no rostral tissues above the midbrain. Case 2 was a 5-year-old boy in whom CT showed retention of the thalamus. Short latency somatosensory evoked potentials (SSEP) in both cases exhibited the absence of cortical activity (N1 and P4) with the preservation of waves of brain stem origin. However, in case 1, wave component No was not observed, while No was seen in case 2. It was postulated, thus, that the No component of SSEP on median nerve stimulation in children, which corresponds to N16 in adults, may originate in the thalamus.
Child, Preschool↗
Human plasma gelsolin reversibly binds Mg-ATP in Ca(2+)-sensitive manner.
Gelsolin is a Ca(2+)-regulated actin-modulating protein found in a variety of cellular cytoplasm and also in blood plasma. Affinity separation of human plasma gelsolin was successfully accomplished by eluting the protein with a low concentration of nucleoside polyphosphate from immobilized Cibacron Blue F3GA (1, 2). This finding was followed by the demonstration that the protein had one class of ATP binding site with Kd = 2.8 x 10(-7) M, which saturated at an ATP/gelsolin ratio of 0.6 in the absence of Ca2+ (3). To obtain further information on the nucleotide binding properties of gelsolin, binding studies were done in the presence of EGTA with GTP, ADP, and GDP by equilibrium dialysis. Incubation of plasma gelsolin with GTP resulted in binding of 0.6 mol of GTP per mol of protein with a dissociation constant of 1.8 x 10(-6) M, indicating that ATP binds to gelsolin with higher affinity than GTP. Neither ADP nor GDP at up to 100 microM appreciably bound to gelsolin at a physiological salt concentration. Then, the effects of divalent metal ions on the ATP binding to plasma gelsolin were examined. Gelsolin bound to ATP with Kd = 2.4 x 10(-6) M in a solution containing 2 mM MgCl2, whereas micromolar free Ca2+ concentrations inhibited ATP binding. Furthermore, addition of Ca2+ rapidly reversed the preformed nucleotide binding to gelsolin, suggesting that Ca2+ binding to gelsolin leads to a conformational change which disrupts a nucleotide binding fold in the protein molecule.
Adenine Nucleotides↗
cDNA cloning and tissue distribution of a rat ubiquitin carboxyl-terminal hydrolase PGP9.5.
We have isolated a cDNA clone encoding ubiquitin carboxyl-terminal hydrolase PGP9.5 from a rat brain cDNA library and examined the tissue distribution. The primary structure of the cDNA consists of 856 nucleotides including the entire coding region for 223 amino acids, and the calculated molecular mass is 24,782 Da. The rat PGP9.5 is strikingly homologous to the human PGP9.5, 75.2% of nucleic acids and 95.1% of amino acids being identical. The mRNA of PGP9.5 is most abundant in the rat brain and to a lesser degree in the testis. In other peripheral tissues we tested, the mRNA was undetectable. Western blotting using an anti-rat PGP9.5 antibody revealed the parallel distribution of mRNA and protein in various brain regions and testis. The availability of the rat PGP9.5 clone provides a new approach to examine the function of PGP9.5 and the role that it plays in the pathology of neurodegenerative diseases.
Amino Acid Sequence↗
Cooperative transcription activation between Ad1, a CRE-like element, and other elements in the CYP11B gene promoter.
We previously reported the presence of six different cis-acting elements (Ad1 to Ad6) in the promoter region of the bovine CYP11B gene. Although the Ad1 site (TGACGTGA) was similar to a palindromic CRE (TGACGTCA), two other upstream sequences, Ad3 and Ad4, were identified as the cAMP response sequences of the gene. We analyzed the functional relationship between the Ad1 site and the upstream elements. Mutation analyses of the Ad1 site indicated that the 5' half of the site (TGACG) was important for the transcription of the gene in vitro. In Y-1 cells, a plasmid with a mutated Ad1 showed no response to cAMP. The effect of the mutation at the Ad1 site on the cAMP response was almost the same as that of the deletion of Ad3 and Ad4, although the role of each element seemed to be different. These results indicated that both the Ad1 site and the upstream elements, Ad3 and Ad4, were necessary for the full response to cAMP of the CYP11B gene. When the Ad1 site in the promoter region was replaced with a palindromic CRE, elevated transcription activity was detected both in vitro and in vivo. Two kinds of CREBs (43 and 47 kDa) purified from a HeLa cell nuclear extract bound to the Ad1 site. The binding of the palindromic CRE to the nuclear factor(s) was stronger than that of Ad1.
Animals↗
The treatment of osteoporotic-posttraumatic vertebral collapse using the Kaneda device and a bioactive ceramic vertebral prosthesis.
Twenty-two patients with neurologic deficit due to delayed posttraumatic vertebral collapse after osteoporotic compression fractures of the thoracolumbar spine underwent anterior decompression and reconstruction with bioactive Apatite-Wollastonite containing glass ceramic vertebral prosthesis and Kaneda instrumentation. Eighteen patients previously had minor trauma that resulted in a mild vertebral compression fracture without any neurologic involvement and were either conservatively treated or not treated at all. Four had no history of back injury. The preoperative neurologic status was incomplete paralysis in all patients. The average age at surgery was 66 (53-79) years. The average follow-up was 34 (20-58) months after surgery. All patients had returned to their daily living with neurologic recovery and stable spine. This type of anterior procedure is effective in the osteoporotic patients and there was a very low incidence of instrumentation failure and very low morbidity.
Aged↗
Involvement of calcium in germination of coat-modified spores of Bacillus cereus T.
The effect of calcium on germination of coat-modified Bacillus cereus T spores was investigated. Coat-modified spores produced either by chemical extraction (SDS-DTT-treated spores) or by mutagenesis (10LD mutant spores) were unable to germinate in response to inosine. While SDS-DTT-treated spores could germinate slowly in the presence of L-alanine, 10LD mutant spores could not germinate at all. The lost or reduced germinability of coat-modified spores was restored when exogenous Ca2+ was supplemented to the germination media. The calcium requirement of coat-modified spores for germination was fairly specific. The simultaneous presence of germinant with Ca2+ was also required for germination of coat-modified spores. The optimal recovery of germinability was observed in the presence of 1.0 mM of calcium acetate. The calcium requirement itself was remarkably diminished under the condition in which L-alanine and a certain purine nucleoside analog, adenosine or inosine, coexisted. The lost or diminished germinability observed in SDS-DTT-treated spores or 10LD mutant spores may be attributed to the loss of calcium associated with the spore integuments.
Alanine↗
Recurrent annular erythema associated with anti-SS-B/La antibodies: analysis of the disease-specific epitope.
We have found that anti-SS-B/La antibodies are present in a group of patients with a characteristic recurrent annular erythema, and immunological abnormalities. The presence of a disease-specific epitope for this entity has been examined by comparing immunological reactivity of anti-SS-B/La antibodies between these patients and patients with classical Sjögren's syndrome who have anti-SS-B/La antibodies but do not have this characteristic erythema. In immunoblotting using three different antigen sources, all the sera from both groups exhibited the same reaction patterns. V8 protease mapping showed that the sera could be divided into three groups by the difference in reactivity to two main digested peptide fragments. However, no significant relationship of the reactivity in peptide mapping between the two patient groups was observed. Further studies are necessary to elucidate the role of anti-SS-B/La antibodies in disease pathogenesis in these patients, in particular by more precise peptide mapping using recombinant SS-B/La antigen proteins.
Adult↗
Sporadic medullary microcarcinoma of the thyroid.
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Adenoma↗
Carcinoma of the thyroid at a young age--a review of 23 patients.
Twenty-three children and adolescents with thyroid carcinoma who were 20 years old or younger at the time of initial treatment were followed for a mean time of 9.5 years. Of the tumours, 21 were papillary and two follicular carcinomas. The majority (91%) of the 23 cases showed extra-thyroidal extension of the tumour. An association with chronic thyroiditis was observed in 30% of the cases. There was no history of irradiation of the head and neck in any patients. Relapse occurred in eight patients, lung metastases in two and local recurrences in six; one patient died from lung metastases 3 months after initial treatment. Male patients, advanced stage of tumour, less differentiated histological features and DNA aneuploidy were associated with a higher frequency of relapse. The prognosis of thyroid carcinomas in children and adolescents is not so good as is generally believed.
Adenocarcinoma↗
Papillary thyroid carcinoma in Kanazawa, Japan: prognostic significance of histological subtypes.
A retrospective analysis of 183 papillary thyroid carcinomas was made in order to assess the prognostic factors related to survival. The following factors were found to adversely affect the prognosis: trabecular subtype of papillary carcinoma, the extent of the primary tumour, regional lymph node involvement, the presence of distant metastases, old age, male sex and the extent of the neck dissection. The presence of the follicular variant of papillary carcinoma and the extent of the thyroidectomy did not influence the prognosis. The trabecular subtype of papillary carcinoma is characterized by a trabecular or solid arrangement of follicular cells with nuclei of ground-glass appearance. Univariate and multivariate analysis indicated that patients with this type of thyroid tumour had a poorer prognosis than those with the well-differentiated or follicular variants of papillary carcinoma. In our opinion the trabecular subtype of papillary carcinoma should be included as a separate entity in the WHO classification of thyroid tumours.
Adolescent↗
Papillary carcinoma of the thyroid gland with fibromatosis-like stroma.
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Aged↗
Histologic changes in Graves' thyroid gland after 131I therapy for hyperthyroidism.
The effect of 131I therapy was examined in 13 thyroid glands affected by Graves' disease 3 to 29 years after irradiation for hyperthyroidism. All of the patients had clinically palpable thyroid nodules. Two patients were in a latent hypothyroid, 6 in a euthyroid and 5 in a hyperthyroid state. The microscopic changes in the thyroids showed a pattern of multiple adenomatous nodules with cystic changes, marked oxyphilic cell changes with nuclear atypism and various degrees of chronic thyroiditis. Immunohistochemical staining for TG and T4 was negative to mildly positive in these oxyphilic cells and entirely negative for EGF and CEA. The DNA ploidy pattern was diploid pattern in 6 cases. One papillary-type microcarcinoma occurred, but there was no evidence of a relationship between the tumor and the irradiation. The pathologic findings in Graves' thyroid gland after 131I therapy are not specific, but pathologists should differentiate this lesion from adenomatous goiter, which occurs with no apparent cause, or from thyroid carcinoma because of the marked nuclear atypism of this lesion.
Adult↗
Prenatal diagnosis in high risk pregnancies for Zellweger syndrome.
Zellweger syndrome is a lethal disorder. At present, no effective therapies are known for the patients of Zellweger syndrome. Recently a typical case of Zellweger syndrome in Japan was observed. In spite of intensive care, the patient died at the age of 3 months. Following this, the parents requested prenatal diagnosis for their following two pregnancies. We investigated levels of very long chain fatty acids (VLCFA), levels of bile acids in amniotic fluid and immunoblotting of peroxisomal beta-oxidation enzymes in cultured amniocytes. We report that immunoblotting using cultured amniocytes is an effective method for prenatal diagnosis of Zellweger syndrome. Furthermore, if we use immunoblotting for prenatal diagnosis, we can discriminate pseudoZellweger syndrome from pseudoneonatal adrenoleucodystrophy. Following prenatal diagnosis, two healthy babies were delivered. After birth, no abnormal levels of VLCFA in either serum or red blood cell membranes were confirmed. In this paper, we report that we can diagnose a healthy fetus in a high risk pregnancy for Zellweger syndrome.
Amniocentesis↗
Protein kinase C-independent sensitization of contractile proteins to Ca2+ in alpha-toxin-permeabilized smooth muscle cells from the guinea-pig stomach.
Involvement of protein kinase C in receptor-operated Ca2+ sensitization of cell shortening was investigated by use of alpha-toxin-permeabilized smooth muscle cells from the fundus of the guinea-pig. Most of the isolated cells responded to 0.6 microM Ca2+ with a maximal shortening to approximately 65% of the resting cell length. Addition of acetylcholine (ACh) at a maximal concentration (10 microM) resulted in a marked decrease in the concentration of Ca2+ required to trigger a threshold response from 0.6 microM to 0.2 microM. The augmentation of Ca2+ sensitivity by ACh was not inhibited by specific protein kinase C inhibitors, calphostin C and K-252b at a concentration of 1 microM. These findings suggest that protein kinase C is not involved in the muscarinic receptor-operated augmentation of Ca2+ sensitivity.
Acetylcholine↗