GS Type Research Articles

GRISCELLI SYNDROME TYPE 2 –CLINICAL APPROACH AND CASE REPORT

Griscelli syndrome type 2 is rare authosomal ressesive disorder caused by a defect in the RAB27A gene, which affects a melanosome-anchoring complex in melanocytes, affecting release of cytolytic granules from T and NK cells. Children with GS type 2 develop an uncontrolled T-lymphocyte and macrophage activation syndrome known as hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH). We describe a 3 years old girl patient classic features of Griscelli syndrome type 2 A 3-year-old girl was admitted to hematology with complaints of LAP, hepatosplenomegaly and pancytopenia (WBC- 3080/µl, Hb-7.6 g/dl, Neutr.-350/µl, PLT - 165000/µl). The patient's condition was below the percentile, her skin was bronze, her hair was silver-grey. HLH criteria were met (triglycerides 458 mg/mL, ferritin 3445 ng/mL, fibrinogen 180 mg/dl). Morphology of the bone marrow was hypocellular, signs of dyserythropoiesis (stage I) and megakaryocytes were reduced According to the clinical and laboratory data(hepatosplenomegaly, increased ferritin, hypertriglyceridemia, pancytopenia, hyperthermia resistant to antimicrobial therapy, silver-gray hair, pigment balls of hair seen light microscope) and the death of another undiagnosed child in the family, suggested likely primary HLH and GS. As a result of genetic analysis (homozygous mutation c.514_ 518delCAAGC(p.GLN172Asnfs*,rs767481076)1 in the RAB27A gene), the diagnosis of GS type 2 was confirmed. The patient was treated according to the HLH 2004 protocol. CSA levels were measured once a week. IVIG support was given based on IgG levels. HSCT was planned from patient's healthy HLA-matched sibling, but HSCT was delayed because the brother was infant. After 45 weeks of maintenance therapy, etoposide was discontinued, dose of dexamethasone was reduced to 5 mg/kg, but CSA was continued at the same dose. Control studies are carried out once a week. As far as possible HSCT is planning The prognosis of patients with Griscelli syndrome is poor. It is usually rapidly fatal within 1-4 years without aggressive treatment and bone marrow transplantation at onset of an accelerated phase. HSCT is more successful when implemented early course of the disease. Palliative care includes treatment and prophylaxis care infections and immunosupressor therapy in accelerated phases. Some patients have died after transplantation, but others have had lasting remissions

Study of Organosolv Lignins by Methods of FTIR and NMR Spectroscopy

The analysis of the literature on the spectral methods of lignins study was carried out. The most suitable spectral methods for elucidating the structural features of soluble organosolv lignins were selected. The possibilities of FTIR, 1Н NMR, 13С NMR and 2D HSQC NMR methods were demonstrated on the example of studying the structure of ethanollignins obtained from fir and aspen wood. It was established that fir and aspen lignins are G type and GS type lignins, respectively. The HSQC spectra analysis demonstrated a presence of β–O–4′, β–β′, β–5′ linkages in the ethanollignins. It was found that β–O–4′ linkages in ethanollignins from fir and aspen were partially acylated with ethoxy groups in the α-position

Antihypertensive drugs

Antihypertensive drugs

Characterization of lignin in heartwood, sapwood and bark from Tectona grandis using Py–GC–MS/FID

Sapwood, heartwood and bark of 70-year-old teak trees from an unmanaged stand in East Timor were characterized by pyrolysis (Py–GC–MS/FID). Sapwood and heartwood from teak presented a high lignin content (35.4 and 37.3 % of extractive-free wood) and similar composition. Teak wood is characterized by a GS type of lignin (56.0 % of G-units, 42.2 % of S-units and 1.8 % of H-units in extractive-free wood), with an S/G ratio of 0.8. The major lignin-derived compounds were 4-vinylsyringol (6.6 %), coniferyl alcohol (trans) (4.5 %), coniferaldehyde (2.0 %), 4-vinylguaiacol (1.6 %) and 4-methylguaiacol (1.6 %). Extractive-free teak bark presented 28.0 % of lignin, characterized by a GS type, with 53.3 % G-units, 42.1 % S-units and 4.6 % H-units, with S/G ratio of 0.8. The high lignin content and a composition with predominance of G-units may contribute to the durability and mechanical resistance of teakwood.

트롤어구용 해파리 분리 배출장치 기본 구조 개발

The purpose of this study is to develop the jellyfish separator system(JSS) for reducing fishery damage by the increase of jellyfish in the sea area of Korea in summer. First of all, to find the optimum structure of a JSS, six types of JSS in trawl fishery were designed and manufactured, the underwater shape of JSS and the separating process by JSS were observed in the circulating water channel(CWC). And the field experiments were carried out in July and September 2004 in the southern sea of Korea. For the moving path of the jellyfish model in the CWC, in case that the model was larger than the mesh size of the separator net, it was guided toward the lower part of the separator net by the guiding net and discharged through the outlet. In case that the model was smaller than the mesh size of the separator net, some models which passed through the upper part of the guiding net were smaller than the mesh size of the guiding net and discharged through the outlet and most of the model which passed through the lower part of the guiding net moved to the codend passing through the separator net. According to the field experiment result, the optimum tilt angle of separator net was inferred <TEX>$20^{\circ}$</TEX> that the discharge rate of jellyfish was higher than the other tilt angle of separator net and the optimum structure of JSS was inferred GS type(consists of guiding net and separator net) that the discharge rate of jellyfish was higher than S type(consists of separator net). It was demamded to carry out more study for the countermeasure to reduce loss of fish.

Development of in situ melanoma after allogeneic bone marrow transplantation in Griscelli syndrome type II

GS is an uncommon autosomal recessive disorder characterized by pigmentary dilution of the skin and hair and in most patients by abnormal regulation of the immune system. Childhood melanoma is rare in the pediatric population. The best prognosis is achieved with early diagnosis and definitive surgical excision of melanoma. We report a case of a patient with GS type II and melanoma who was successfully treated by allogeneic bone marrow transplantation and surgical excision of the melanoma.

The ammonium‐inactivated cyanobacterial glutamine synthetase I is reactivated in vivo by a mechanism involving proteolytic removal of its inactivating factors

The Synechocystis sp. PCC 6803 glutamine synthetase type I (GS) activity is controlled by a process that involves protein-protein interaction with two inactivating factors (IF7 and IF17). Following addition of ammonium, the genes encoding these proteins, gifA and gifB, respectively, are derepressed, leading to the synthesis of IF7 and IF17 and consequently GS is inactivated. Upon ammonium removal, the GS activity rapidly returns to the initial level within 20 min. In this study, we analyse the mechanism underlying GS reactivation and find that this process involves IF7 and IF17 degradation. We show that the presence of ammonium as nitrogen source enhances IF17 but not IF7 stability independently of gif gene transcription. Studies with Synechocystis crude extracts under different conditions revealed that IF7 and IF17 display different stabilities in vitro. We found that IF7 is degraded in vitro by the activity of metalloproteases. Furthermore, the involvement of soluble processing metallopeptidases in IF7 degradation has also been demonstrated in vivo, by analysing Synechocystis mutant strains devoid of genes of the prp family. Finally, using a Synechocystis strain lacking GS type I, we establish the crucial role of the target protein GS for in vivo IF7 and IF17 stability.

운전자의 불안전한 행위와 성격유형과의 상호관계에 관한 연구

The goal of this study is to find out correlation between Driver's Unsafe acts(errors and violations) and Personality types. The experiment was performed on 180 subjects, men and women between 20's and 60's having experience in driving for 6 months at least. Personality types of the subjects were classified by MBTI(Myers-Briggs Type Indicator) GS type and Driver's unsafe acts were measured by KDBQ(Korean Driver Behavior Questionnaire) based on Reason's DBQ(Driver Behavior Questionnaire). The result of experiment showed several facts about the relation. The first is that the drivers of P (Perceiving) type commit more violations and slips than drivers of J(Judging) type. The second is that in the comparison among attitude indexes(EP, EJ, IP, IJ) the drivers of EP(Extroversions - Perceiving) commit more violations than other type drivers. Finally, only men of P(Perceiving) type commits more violations than men of J(Judging). Based on these facts, it is possible to use Personality types as a device to prevent unsafe acts in various fields for driver selection and accident prevention training classified by Personality types etc.

Prostaglandin E(2) inhibits calcium current in two sub-populations of acutely isolated mouse trigeminal sensory neurons.

Prostaglandins are important mediators of pain and inflammation. We have examined the effects of prostanoids on voltage-activated calcium currents (I(Ca)) in acutely isolated mouse trigeminal sensory neurons, using standard whole cell voltage clamp techniques. Trigeminal neurons were divided into two populations based on the presence (Type 2) or absence (Type 1) of low voltage-activated T-type I(Ca). The absence of T-type I(Ca) is highly correlated with sensitivity to mu-opioid agonists and the VR1 agonist capsaicin. In both populations of cells, high voltage-activated I(Ca) was inhibited by PGE(2) with an EC(50) of about 35 nM, to a maximum of 30 %. T-type I(Ca) was not inhibited by PGE(2). Pertussis toxin pre-treatment abolished the effects of PGE(2) in Type 2 cells, but not in Type 1 cells, whereas treatment with cholera toxin prevented the effects of PGE(2) in Type 1 cells, but not in Type 2 cells. Inhibition of I(Ca) by PGE(2) was associated with slowing of current activation and could be relieved with a large positive pre-pulse, consistent with inhibition of I(Ca) by G protein betagamma subunits. Reverse transcription-polymerase chain reaction of mRNA from trigeminal ganglia indicated that all four EP prostanoid receptors were present. However, in both Type 1 and Type 2 cells the effects of PGE(2) were only mimicked by the selective EP(3) receptor agonist ONO-AE-248, and not by selective agonists for EP(1) (ONO-DI-004), EP(2) (ONO-AE1-259) and EP(4) (ONO-AE1-329) receptors. These data indicate that two populations of neurons in trigeminal ganglia differing in their calcium channel expression, sensitivity to mu-opioids and capsaicin also have divergent mechanisms of PGE(2)-mediated inhibition of calcium channels, with Gi/Go type G proteins involved in one population, and Gs type G proteins in the other.

GSn: A new service type for integrated services on the internet

AbstractNetwork services with deterministic guarantees are based on a worst—case description of user—generated traffic. When designing a policing and scheduling algorithm for guaranteed services on the Internet, accuracy of description of the traffic profile has to be traded with simplicity of implementation. The result of this trade off is often expressed as the number of token buckets required by the service along with the choice of their parameters. The GS type of service proposed by the IETF uses two token buckets both for characterizing the traffic and for policing it. The choice of using only two token buckets is primarily driven by policing costs. In this paper we propose a method that allows the number of token buckets used for characterizing the traffic to be greater than what is actually needed to police it. This means we can obtain an accurate profile of the traffic while keeping policing simple. The method consists of computing a profile of the traffic which involves a number of token buckets of the order of ten, and then doing the policing using only the first token bucket, plus another one which is chosen depending on the delay requirements of the receivers. This paper shows that with this simple enhancement we obtain a guaranteed service whose performance closely approaches the theoretical limits of services with deterministic guarantees.

Nitrogen control of the glnN gene that codes for GS type III, the only glutamine synthetase in the cyanobacterium Pseudanabaena sp. PCC 6903.

Pseudanabaena sp. strain PCC 6903 is the first cyanobacteria lacking the typical prokaryotic glutamine synthetase type I encoded by the glnA gene. The glnN gene product, glutamine synthetase type III, is the only glutamine synthetase activity present in this cyanobacterium. Analysis of glnN expression clearly indicated a nitrogen-dependent regulation. Pseudanabaena glnN gene expression and GSIII activity were upregulated under nitrogen starvation or using nitrate as a nitrogen source, while low levels of transcript and activity were found in ammonium-containing medium. Primer extension analysis showed that the glnN gene promoter structure resembled that of the NtcA-related promoters. Mobility shift assays demonstrated that Synechocystis sp. PCC 6803 NtcA protein, expressed and purified from Escherichia coli, bound to the promoter of the Pseudanabaena 6903 glnN gene. The NtcA control of the glnN gene in this cyanobacterium suggested that, in the absence of a glnA gene, NtcA took control of the only glutamine synthetase gene in a fashion similar to the way the glnA gene is governed in those cyanobacteria harbouring a glnA gene.

Developmental expression of G-proteins and adenylyl cyclase in peripheral olfactory systems. Light microscopic and freeze-substitution electron microscopic immunocytochemistry.

Light microscopic immunohistochemistry coupled with freeze-substitution electron microscopic immunocytochemistry was used to localize alpha-subunits of G-proteins and type III adenylyl cyclase in developing rat olfactory epithelia. Some cilia immunoreacted with antibodies to GS alpha and type III adenylyl cyclase as early as prenatal day 15 (E15; E1 = sperm-positive), but immunolabelling with antibodies to Golf alpha was not observed until E16. From then on numbers of receptor cells with immunolabelled cilia increased for all three probes. Immunoreactivity for antibodies to the olfactory signal-transduction proteins tended to parallel cilium development, though Golf alpha lags somewhat behind. Newly formed cilia labelled along their lengths, whereas mature cilia labelled predominantly along their long distal parts. Dendritic knobs and ciliary necklaces showed little or no labelling. While at E22 most multiciliate cells immunolabelled with antibodies to Gs alpha, Golf alpha, and type III adenylyl cyclase, not all of these cells labelled with antibodies to olfactory marker protein. Olfactory axons immunoreacted more intensely than epithelial surface structures with antibodies to Gs alpha at E15; the reverse occurred by about E18. Immunoreactivity with antibodies to alpha-subunits of the G-proteins Go, Gq/G11, and Gi was also found as early as E15. Antibodies to Go alpha labelled receptor cell dendritic knobs and cilia during development only. Antibodies to Gi alpha labelled Bowman's glands, whereas those to Gq alpha/G11 alpha bound to receptor cell cilia and axons (primarily vomeronasal), and supporting cell microvilli. We propose that Gs is the predominant G protein in cilia of immature olfactory receptor cells, while Golf is predominant in cilia of mature cells. Axonal immunoreactivity for some G-protein antibodies suggests G-protein participation in processing of olfactory axon and/or axon terminal-bound signals.

Genomic polymorphism, growth properties, and immunologic variations in human herpesvirus-6 isolates

Fifteen human herpesvirus-6 (HHV-6) isolates from normal donors and patients with AIDS, systemic lupus erythmatosis, chronic fatigue syndrome, collagen-vascular disease, leukopenia, bone marrow transplants, Exanthem subitum (roseola), and atypical polyclonal lymphoprolfferation were studied for their tropism to fresh human cord blood mononuclear cells, growth in continuous T cell lines, reactivity to monoclonal antibodies, and by restriction enzyme banding patterns. All isolates replicated efficiently in human cord blood mononuclear cells, but mitogen stimulation of the cells prior to infection was required. The ability to infect continuous T-cell lines varied with the isolates. Isolates similar to GS prototype infected HSB2 and Sup T1 cells and did not infect Molt-3 cells, whereas isolates similar to Z-29 infected Molt-3 cells but not HSB2 and Sup Ti cells. Some of the monoclonal antibodies directed against the HHV-6 (GS) isolate showed reactivity with all isolates tested, but others only reacted with HHV-6 isolates similar to the GS isolate and not with those similar to Z-29 isolate. Restriction enzyme analysis using EcoRI, BamHI, and HindIII revealed that HHV-6 isolates from roseola, bone marrow transplant, leukopenia, and an HIV-1-positive AIDS patient from Zaire (Z-29) were closely related but distinct from GS type HHV-6 isolates. Based on the above findings, we propose that, like herpes simplex virus types 1 and 2, the 15 HHV-6 isolates analyzed can be divided into group A (GS type) and group B (Z-29 type).

Primary afferent projections to the sacral secondary visceral gray: A quantitative electron microscopic examination in the cat

Afferent input to the sacral secondary visceral gray matter was studied by quantitatively assessing synaptic degeneration that follows ipsilateral sacral dorsal rhizotomy. Five days after the lesions, slightly more than 25% of the synaptic contacts showed degenerative changes. The majority of the reactive synapses contained clear spherical vesicles (S type). Boutons containing dense core vesicles (GS type) also showed degenerative changes, and together with S boutons constituted more than 96% of the reactive terminals. Degenerating S type synapses were found only on dendrites whereas GS boutons were observed on both cell bodies and dendrites. Further examination revealed that although there were more S than GS synapses in the nucleus, the percentage of GS terminals affected was greater than that for S type boutons. These results demonstrate a direct primary afferent input to the sacral secondary visceral gray matter utilizing chiefly two different synaptic types.

An ultrastructural examination of dorsal root input to the sacral secondary visceral gray

Dorsal root input to the sacral secondary visceral gray was examined in the adult cat using an orthograde degeneration technique. Five days after unilateral, intradural section of sacral dorsal roots electron-microscopic evidence of synaptic degeneration was seen throughout the nucleus ipsilateral to the side of the lesion. Degenerating sacral primary afferents in this nuclear region were associated mainly with S and GS type boutons. S type synapses showed two distinct degenerative responses. Some of the degenerating boutons were swollen with synaptic vesicles clustered tightly near the presynaptic membrane. Others appear shrunken with consequent packing of vesicles and mitochondria within the presynaptic terminal. Degenerating S terminals were found mainly on dendrites. S type synapses on nerve cell bodies appeared to be unaffected by dorsal root section. The degenerative response of sacral primary afferents associated with GS boutons was characterized by marked swelling of the presynaptic terminal and the presence of significant amounts of glycogen. Degenerating GS boutons were observed on both cell bodies and dendrites. These observations provide morphologic evidence of a direct synaptic link between dorsal root afferents and nerve cells in the sacral secondary visceral gray.

A quantitative ultrastructural analysis of the synaptic population in the sacral visceral gray

The synaptic distribution in the sacral visceral gray of the cat was examined. Synaptic complexes exhibiting a distinct membrane specialization were identified and classified according to vesicle type and number. Boutons containing clear spherical vesicles (S type) account for more than 63% of the synaptic contacts on both cell bodies and dendrites. Terminals containing three or more granular vesicles (GS type) comprise between 23 and 31% of the synapses in the nucleus. F boutons are less frequently seen (3–6%) as are T type boutons (1–2%). Presynaptic terminals containing a predominance of dense core vesicles (GG type) constitute less than 1% of the total synapses in this neuropil and are found only on dendrites. Nerve cell bodies receive approximately five synaptic contacts per 100 μm of postsynaptic membrane. Large and medium size dendrites are contacted by an average of seven boutons per 100 μm of postsynaptic membrane and small dendrites have the highest distribution (approximately 12 per 100 μm). Comparison of the percentage distribution of various types of synapses in this nucleus reveals little variation between cell bodies and dendrites. However, expression of the synaptic population as boutons per 100 μm of available postsynaptic membrane clearly illustrates intranuclear differences in synaptic distribution not seen in percentage type comparisons.

On the systematics of elastic scattering at high and intermediate energy

A model for elastic scattering valid in the intermediate-and high-energy region is proposed. The model includes three kinds of entities: the pomeron, a universal GS pomeron; the reggeons, also universal and of GS type; and the core, a low-energy central real piece required by dispersion relations. The number of free functions and parameters is rather small. Our approach supports naive duality and, in general, agrees with the results of absorptive models.