Unilateral spatial neglect in a child with hemiplegic migraine.
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Adolescent↗
Biomedical subjects
E Hirose
Publications and source records attributed to E Hirose.
Full-sized RanBPM cDNA encodes a protein possessing a long stretch of proline and glutamine within the N-terminal region, comprising a large protein complex.
Previously isolated RanBPM, a Ran-binding protein in the microtubule-organizing center, which had been thought to play a role in Ran-stimulated microtubule assembly, turned out to be a truncated protein. To clarify the function of RanBPM, we cloned the full-sized RanBPM cDNA that encodes a 90 kDa protein, compared to the previously isolated cDNA that encoded a 55 kDa protein. The newly cloned 5' coding region contains a great number of cytidine and guanidine nucleotides, like the CpG island. Thus, full-sized RanBPM cDNA encodes a long stretch of proline and glutamine residues in the N-terminal region. It comprises a protein complex of more than 670 kDa. Ran was detected in this complex when RanBPM and Ran were both ectopically expressed. New antibodies to RanBPM were prepared against three different regions of RanBPM. All of them detected a 90 kDa protein that is predominantly localized both in the nucleus and in the cytoplasmic region surrounding the centrosome, but none of them stained the centrosome. In this context, our previous notion that RanBPM is a centrosomal protein should be discarded. RanBPM is well conserved in the animal kingdom. It may play an important role in uncovering Ran-dependent nuclear events.
Adaptor Proteins, Signal Transducing↗
Cellulose in the house of the appendicularian Oikopleura rufescens.
By electron diffraction analysis, highly crystalline cellulose I beta was found in the house (a special structure in which the tunicate lives) of the appendicularian Oikopleura rufescens. Cellulose microfibrils 20 nm in width were observed in a random array or highly organized with rectangular spacing of 2 to 10 microns in the house. The bundled cellulose microfibrils formed in the inlet filters, which are highly ordered meshwork structures. This paper provides the first account of the existence of cellulose in the house of an appendicularian. Our findings showed that the house and tunic are homologous tissues among the tunicates, and that the common ancestor of the tunicates (ascidians, thaliaceans, and appendicularians) already possessed cellulose-biosynthetic ability.
Animals↗
Catheter ablation of an epicardial accessory pathway via the middle cardiac vein guided by monophasic action potential recordings.
This report describes a case of permanent junctional reciprocating tachycardia (PJRT) that was ablated via the middle cardiac vein, guided by monophasic action potential recording. The patient was a 63-year-old woman who had been suffering from palpitation for 10 years. ECG during palpitation showed a narrow QRS tachycardia with a long RP interval. Electrophysiological study revealed that this tachycardia was an orthodromic reciprocating tachycardia, via an accessory pathway with a decremental property and a long ventriculoatrial interval (130 ms): PJRT. The earliest atrial activation during tachycardia was detected at the junction of the middle cardiac vein with the coronary sinus. Monophasic action potentials were recorded to confirm that the ablation catheter was in contact with the epicardium.
Cardiac Pacing, Artificial↗
Novel G proteins, Rag C and Rag D, interact with GTP-binding proteins, Rag A and Rag B.
Rag A/Gtr1p are G proteins and are known to be involved in the RCC1-Ran pathway. We employed the two-hybrid method using Rag A as the bait to identify proteins binding to Rag A, and we isolated two novel human G proteins, Rag C and Rag D. Rag C demonstrates homology with Rag D (81.1% identity) and with Gtr2p of Saccharomyces cerevisiae (46.1% identity), and it belongs to the Rag A subfamily of the Ras family. Rag C and Rag D contain conserved GTP-binding motifs (PM-1, -2, and -3) in their N-terminal regions. Recombinant glutathione S-transferase fusion protein of Rag C efficiently bound to both [(3)H]GTP and [(3)H]GDP. Rag A was associated with both Rag C and Rag D in their C-terminal regions where a potential leucine zipper motif and a coiled-coil structure were found. Rag C and D were associated with both the GDP and GTP forms of Rag A. Both Rag C and Rag D changed their subcellular localization, depending on the nucleotide-bound state of Rag A. In a similar way, the disruption of S. cerevisiae GTR1 resulted in a change in the localization of Gtr2p.
Amino Acid Motifs↗
Quality of life in kidney transplant patients.
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Adult↗
Mutations in the 11-cis retinol dehydrogenase gene in Japanese patients with Fundus albipunctatus.
PURPOSE: To detect mutations in the RDH5 gene encoding 11-cis retinol dehydrogenase in patients from Japan with fundus albipunctatus. METHODS: Polymerase chain reaction and direct genomic sequencing techniques were used to detect mutations of the RDH5 coding exons (exons 2-5) in two unrelated patients with fundus albipunctatus. Selected alleles that altered the coding region or intron splice sites were evaluated further through segregation analysis in the families of the index cases. RESULTS: Two novel RDH5 mutations were identified. One of these was a missense mutation Val264Gly in exon 5, and the other was an in-frame insertion of 3 bp in exon 5. CONCLUSIONS: The data indicate that mutations in RDH5 are the primary cause of fundus albipunctatus.
Alcohol Oxidoreductases↗
RagA is a functional homologue of S. cerevisiae Gtr1p involved in the Ran/Gsp1-GTPase pathway.
Human RagA and RagB is reported to be 52% identical to a putative GTPase of Saccharomyces cerevisiae, Gtr1p. According to the reported nucleotide sequence, we amplified human RagA and RagBs cDNAs from the human B cell cDNA library with PCR. Both cDNAs rescued a cold sensitivity of S. cerevisiae, gtr1-11. Furthermore, we introduced into the cloned human RagA cDNA, the mutation 'T21L' corresponding to the gtr1-11 mutation which has been reported to suppress not only all of rcc1-, temperature-sensitive mutants of Ran/Gsp1p GTPase GDP/GTP-exchanging factor, but also rna1-1, a temperature-sensitive mutant of Ran/Gsp1p GTPase-activating protein. The resulting RagAgtr1-11 cDNA partially, but significantly, suppressed both rcc1- and rna1-1 mutations. These results indicated that RagA and RagBs are functional homologues of S. cervisiae Gtr1p. Interestingly, while wild-type human RagA and RagBs were localized within the cytoplasm, similar to S. cerevisiae Gtr1p, the mutated human RagAgtr1-11 corresponding to a dominant negative form of RagA was distributed in discrete speckles in the nucleus, being localized side by side with SC-35, a non-snRNP of the splicing complex. In contrast, a dominant positive form of RagA, Q66L was localized in the cytoplasm. Thus, RagA was suggested to shuttle between the cytoplasm and the nucleus, depending on the bound nucleotide state.
Amino Acid Sequence↗
Transgenic medaka fish bearing the mouse tyrosinase gene: expression and transmission of the transgene following electroporation of the orange-colored variant.
Transgenic fish bearing the mouse tyrosinase gene (mg-Tyrs-J) were produced by transfection into fertilized eggs of the homozygous normal orange-colored variant of medaka fish, Oryzias latipes, by means of electroporation. Of 589 eggs transfected, 38 fish (6%) exhibited brownish wild-type skin pigmentation, which was discernible from control siblings. Light microscopy of the skin from the founders thus generated disclosed that 1) melanization occurred and was restricted to melanophores formed presumably from pre-existing amelanotic melanophores, 2) there was a wide variation in the degree of melanization observed among melanophores, and 3) no melanin deposition was recognized in xanthophores or leucophores. Immunofluorescence using an antibody raised against mouse tyrosinase disclosed that melanophores at varying stages of maturation were reactive. Thus, it was shown that the transgene in medaka fish expressed its action in a cell type-specific manner. Crossing of transgenic founders with homozygous orange-colored variant fish yielded two groups of offspring expressing either the wild-type or the orange-colored skin pigmentation at an approximate ratio of 1:1. Crossing between founders exhibiting wild-type pigmentation yielded only offspring with melanized skin. Skin melanophores in these offspring formed vertical stripes, which are rare in this species. The hereditary basis of melanized skin was demonstrated in matings of F1 progenies, which resulted in similar degrees of melanization over whole skin melanophores. The sum of these findings implied that the transgene is expressed as a dominant character gene and is transmitted through germ cell lines according to the Mendelian law. PCR analysis combined with nested PCR technique strongly suggested that the transgene was integrated into the medaka genome, even though the copy number deduced from gel banding was largely diminished, possibly as a result of fragmentation or instability within the medaka genome.
Animals↗
Transfer of the heart pacemaker during juvenile development in the isopod crustacean Ligia exotica.
Developmental changes in heartbeat pacemaker mechanisms were examined electrophysiologically in the isopod crustacean Ligia exotica. The heartbeat of embryos and early juveniles was myogenic. The heart muscle cells were coupled electrically, and no localized pacemaker activity was found in the heart. In newly hatched juveniles, the cardiac ganglion exhibited no spontaneous activity, although stimulation of the cardiac ganglion produced excitatory junctional potentials (EJPs) in the heart muscle. The myogenic activity of the heart was reset and entrained by the EJPs evoked by ganglionic stimulation. During juvenile development, spontaneous EJPs appeared irregularly in the heart muscle. Later in development, the cardiac ganglion started rhythmic bursting, and each muscle response followed a ganglionic burst discharge and overlapped the EJPs evoked by ganglionic activity. At this point, the activity of the cardiac ganglion was suppressed by application of tetrodotoxin (TTX); however, even in old adults, both muscle activity and the heartbeat continued following TTX application. Heartbeat frequency was lower in TTX-containing saline than in normal saline. These results show that, during juvenile development, the heart pacemaker is transferred from the heart muscle to the cardiac ganglion, which becomes the primary pacemaker and entrains the heart muscle activity to a higher frequency via EJPs.
Animals↗
Minimum essential region of CCG1/TAFII250 required for complementing the temperature-sensitive cell cycle mutants, tsBN462 and ts13 cells, of hamster BHK21 cells.
CCG1/TAFII250, the largest subunit of the TFIID complex, is mutated in ts cell cycle mutants of BHK21 cells, ts13 and tsBN462, which have a promoter-selective transcriptional defect. A series of deletion mutants of CCG1 cDNA were prepared and transfected into these mutants, in order to identify functional domains of CCG1 required for the complementation of ts 13/BN462 mutation. We determined the minimum size of CCG1:CCG1ME, essential for complementing the ts mutation, which possessed one proline cluster, an HMG1-like domain, and a nuclear localization signal, but which lacked the bromo domains and the acidic phosphorylation sites for casein kinase II common to transcriptional activators. It encodes a protein of 140 kDa. These characteristics of CCG1ME correspond to yeast TAFII145, the yeast homolog of human TAFII250. CCG1ME bound to TBP, creating its own TFIID complex different from that of the endogenous mutated CCG1 in ts+ transformants of tsBN462 cells.
Animals↗
Allorecognition in compound ascidians.
In botryllids (colonial ascidians), there are two types of allorecognition: colony specificity and colony resorption. Colony specificity is manifested by fusion and rejection between two conspecific colonies. The genetic basis for this colony specificity resides in a single highly polymorphic gene locus (fusibility locus) with codominantly expressed alleles. Two colonies with no alleles in common at this locus reject each other, whereas colonies sharing at least one allele at the fusibility locus fuse and form a chimera. That is, in colony specificity, self components are distinguished from nonself components, and failure to recognize self induces rapid rejection reactions. The process of rejection in colony specificity is not uniform among all botryllid ascidians. Colony resorption can occur after the establishment of fusion between two colonies. Zooids of one partner of a chimera are resorbed more than 1 week after fusion, or, by amputation of fused blood vessels, the chimera becomes separated into the two original colonies. Colony resorption is also controlled mainly by the fusibility locus. It usually occurs in a chimera between two colonies sharing only one allele at this locus. In colony resorption, nonself determinants are recognized and chronic rejection reactions are induced resembling MHC-dependent graft rejection. Based on these findings, the fusibility locus of botryllids seems to be very similar to the MHC of vertebrates. Considering that vertebrates evolved directly from ascidians, it is likely that the fusibility locus is an ancestral form of the vertebrate MHC.
Alleles↗
Deficiency of the gene B impairs differentiation of melanophores in the medaka fish, Oryzias latipes: fine structure studies.
In an orange-colored variant of the medaka fish, Oryzias latipes, which is homozygous for b allele, the melanophores represent a tissue-specific differentiation, manifesting an amelanotic appearance in the skin, an incomplete melanogenesis in the choroid and the peritoneum, and mosaic phenotype-like melano-iridophores in the peritoneum. In a wild-type strain of this species carrying the B gene, all melanophores are terminally differentiated irrespective of the tissues in which they are located. This indicates that the deficiency of B gene impairs the differentiation of melanophores in the medaka. Electron microscopy disclosed that the deficiency of B gene causes deterioration of melanogenesis to occur inside the melanosomes and that the manner of deterioration in the melanophores in the skin, the choroid and the peritoneum is different. The ubiquitous occurrence of reflecting platelet-laden melanophores in the peritoneum of this variant and the total absence of a mosaicism in pigment cells of the wild-type strain indicate that the deficiency of B gene predestines melanoblasts distributed in this tissue to an ambiguous state with regard to their differentiation. Little difference is observed between melanosomes maturation in pigment epithelial cells of the orange-colored variant and the wild-type strain, indicating an innocent role of the B gene in their differentiation.
Animals↗
Dual appearances of pigment cells from in vitro cultured embryonic cells of Japanese flounder: an implication for a differentiation-associated clock.
The cells dissociated from developing embryos of Japanese flounder (Paralichthys olivaceus) are cultured in vitro to examine the developmental fate of their pigment cells in relation to establishment of bilaterally asymmetric integumental coloration in vivo. When neurula embryos are dissociated using trypsin-EDTA in Dulbecco's modified Ca(2+)-, Mg(2+)-free phosphate buffered saline and then cultured in vitro using L-15-based fetal calf serum-supplemented growth medium at 20 degrees C, numerous pigment cells appear twice in the same culture with an interval of approximately 1 month even under similar culture conditions. The first group of pigment cells, which is relatively larger in cell size (about 70 microns wide) and lower in cell density, emerges within 12 hr after plating, whereas the second, which is far smaller in cell size (about 30 microns) and overwhelmingly higher in cell density than the first, does so about 1 month after plating. The timing of their appearances in vitro is in good accordance, respectively, with that observed for the larvae under normal development in vivo; the first group appears at the period corresponding to hatching, whereas the second at the period corresponding to the completion of metamorphosis. Light microscopic examinations disclose that each group of pigment cells is composed of black melanophores and reflecting leucophores, and that the population density of melanophores and leucophores in the first group at the climax of appearance is approximated as 1:4. Typical xanthophores that are distributed in the skin of the larvae of this species are scarcely observed in culture in vitro.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Postnatal development of mRNA specific for a metabotropic glutamate receptor in the rat brain.
We examined the ontogenesis of a subtype of metabotropic glutamate receptors, termed mGluR1, which is linked to phosphoinositide metabolism, in various regions of rat brain during neonatal development. Northern blot analyses of mGluR1 mRNA indicated that mRNA increased monotonously or remained at plateau levels during the first 5 weeks after birth. In situ hybridization analyses supported this conclusion. The result is in contrast with the reported development of the activity in excitatory amino acid-stimulated phosphoinositide turnover during the same period. The latter increases during the first few weeks and then decreases sharply.
Animals↗
Expression and transmission of wild-type pigmentation in the skin of transgenic orange-colored variants of medaka (Oryzias latipes) bearing the gene for mouse tyrosinase.
Transgenic fish carrying a reconstructed mouse tyrosinase gene, mg-Tyrs-J, were produced by microinjecting the gene into the oocyte nucleus of an orange-colored variant of medaka (Oryzias latipes). Of 64 oocytes microinjected and subsequently inseminated, 13 embryos developed normally beyond hatching and three of them exhibited brown skin pigmentation in the adult as was commonly observed in the wild type of this species. Light and electron microscopic examination disclosed a ubiquitous distribution of typical melanophores in the skin of these transgenic fish. Judging from their population density and distribution pattern, it was presumed that melanogenesis in these fish was elicited in amelanotic melanophores that resided in the skin of the orange-colored fish of this variant. Immunofluorescence with use of the anti-mouse tyrosinase antiserum lacking reactivity to medaka tyrosinase clearly disclosed that the gene introduced was expressed in the melanophores of transgenic fish. Crosses of female transgenic fish and males from an orange-colored variant yielded offspring exhibiting wild-type or orange-colored pigmentation in a ratio of 1:1, thus implying that mg-Tyrs-J integrated into the medaka genome behaves like a dominant gene. Little melanogenesis was observed in xanthophores, leucophores and iridophores in transgenic fish, suggesting possible specificity in recognition of teleostean cell types (i.e., melanophores) by the regulatory region of the mouse tyrosinase gene.
Animals↗
3,5-Dihydroxyphenyl-glycine: a potent agonist of metabotropic glutamate receptors.
An amino acid, 3,5-dihydroxyphenylglycine (DHPG) induced current responses in Xenopus oocytes expressing a metabotropic glutamate receptor clone mGluR1. Apparent EC50 of DHPG for mGluR1 was slightly lower than that of (+-)-1-aminocyclopentane-trans-1,3-dicarboxylic acid (ACPD). DHPG responses were partially inhibited by 2-amino-3-phosphonopropionic acid (AP-3). DHPG had no effect on ionotropic glutamate receptors whose expression was induced in the oocytes following injection of poly(A)+ mRNA of rat brains. In hippocampal slices, DHPG produced slow excitation of pyramidal cells, resulting from a depression of Ca(2+)-dependent K+ current and a voltage-dependent K+ current. These results indicate that DHPG is a specific and potent agonist of mGluRs.
2-Amino-5-phosphonovalerate↗
[Prevention of infection in patients with traumatic shock].
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Humans↗