NIH Rare Diseases : 50 glutamate formiminotransferase deficiency is an inheritedmetabolic disorder that affects physical and mental development. there are two forms of this condition, a mild form and a severe form. people with the mild form have minor delays in physical and mental development and may have mild intellectual disability. they also have unusually high levels of a molecule called formiminoglutamate (figlu) in their urine. individuals with the severe form have profound intellectual disability, delayed development of motor skills (sitting, standing, and walking) and megaloblastic anemia. in addition to figlu in their urine, they have elevated amounts of certain b vitamins (called folates) in their blood. glutamate formiminotransferase deficiency is caused by mutations in the ftcd gene. it is inherited in an autosomal recessive pattern. there are currently no treatments known to prevent or delay the signs and symptoms of glutamate formiminotransferase deficiency. last updated: 8/10/2016 ...

GLUTAMATE FORMIMINOTRANSFERASE DEFICIENCY description, symptoms and related genes. Get the complete information in our medical search engine for pheno

Human formiminotransferase cyclodeamidase (FTCD) is an approximately 60 kDa, liver specific, bifunctional enzyme, involved in both histidine and folate metabolism. It is identical with the so-called liver cytosol type 1 antigen (LC1), and has been reported to oligomerize in the cytoplasm. DIARECT™ antigens are for further manufacturing or research use only. ...

The thermal diffuse scattering (TDS) correction developed by Popa & Willis [Acta Cryst. (1997), A53, 537-545] for single-crystal pulsed neutron diffraction is examined via a series of experimental data sets collected at the spallation neutron source IPNS and by comparison with benchmark gamma-ray data. In all examples that have been considered, application of the TDS correction led to appreciable changes in the thermal parameters and has increased the information content of the data at hand by enabling recognition of the achieved degree of accuracy from the pattern of comparative results ...

TY - JOUR. T1 - Heat effects on the retina. AU - Piyathaisere, Duke V.. AU - Margalit, Eyal. AU - Chen, Shih Jen. AU - Shyu, Jeng Shyong. AU - DAnna, Salvatore A.. AU - Weiland, James D.. AU - Grebe, Rhonda R.. AU - Grebe, Lynnea. AU - Fujii, Gildo. AU - Kim, Sahng Yeon. AU - Greenberg, Robert J.. AU - De Juan, Eugene. AU - Humayun, Mark S.. PY - 2003/1/1. Y1 - 2003/1/1. N2 - BACKGROUND AND OBJECTIVE: To study the heat and power dissipation effect of an intraocular electronic heater on the retina. The determination of thermal parameters that are nonharmful to the retina will aid in the development of an implantable intraocular electronic retinal prosthesis. MATERIALS AND METHODS: In dogs, five different retinal areas were touched with a custom intraocular heater probe (1.4 X 1.4 X 1.0 mm) for 1 second while the heater dissipated 0 (control), 10, 20, 50, or 100 mW. In a second protocol, the heater was mechanically held in the vitreous cavity while dissipating 500 mW for 2 hours while monitoring ...

A three-compartment model is presented that describes temperature measurements of tethered flying blowflies, obtained by thermal imaging. During rest, the body temperature is approximately equal to the ambient temperature. At the start of flight, the thorax temperature increases exponentially with a time constant of 30 s; in steady flight, a temperature of approximately 30°C is reached (ambient temperature approximately 25°C). After flight, the temperature of the thorax decreases exponentially with a time constant of 50 s. Fitting the time courses of the three body compartments, i.e. head, thorax and abdomen, with the model allows the thermal parameters to be calculated. The metabolic heat produced by a blowfly during tethered flight is estimated to be approximately 23 mW. ...

The new Aluprof MB-86 window and door series have been designed to offer outstanding insulation properties. It meets the increasing requirements from the legislative and general market demands for the enhanced energy saving construction of new windows and door systems, the standard for this frame is: 77 mm, for the sash: 77 mm (door) or 86 mm (windows). In Order to get the best thermal parameters its best to use wide glass pane packets, that in this system can have the thickness of 67 mm. Offered in three varieties ST, SI and AERO it is the first aluminum system to employ silica aerogel, the nanoporous material that has a very high proportion of free void volume compared to conventional solid materials. Its high pore volume, low solid content, and torturous path amorphous structure give rise to low values of thermal conductivity. Therefore the system features the industry leading thermal performance.. Sehen Sie! » ...

By sequestering the common lithium counterion PF6- in a supramolecular complex, chemists have found they can enhance the cations conductivity and how much current it generates-a finding that could lead to better lithium-ion batteries. MITs Jeremiah A. Johnson, Yang Shao-Horn, and Bo Qiao came up with the idea after a talk Qiao gave during his first week as a postdoc at MIT. Qiao spoke about his doctoral work with cyanostar, a macrocycle that forms a supramolecular complex with PF6- in a 2:1 ratio (shown). LiPF6 is one of the most common salts used as a Li+ source in Li batteries, Johnson says. The chemists realized that if they could bind the anion of that salt with cyanostar, it could dramatically affect the properties of LiPF6. They reasoned that capturing the counterion would spur the salt to break up into free ions and that the large supramolecular complex would diffuse slowly compared with free Li+. Experiments bore out this hypothesis (J. Am. Chem. Soc. 2018, DOI: ...

The development of a novel electrochemically stable host-guest supramolecular complex at a host surface is described. It was constructed by combining a self-assembled monolayer (SAM) of mono-(6-deoxy-6-mercapto)-beta-cyclodextrin (beta CDSH), iron (III) tetra-(N-methyl-4-pyridyl)-porphyrin (FeTMPyP) as the guest-link-molecule and beta-cyclodextrin-functionalized gold nanoparticles (beta CDAuNP). The building process of the layer-by-layer assembly was monitored by surface plasmon resonance spectroscopy (SPR). The binding processes between the host-functionalized gold surface and the linker molecule (FeTMPyP) were verified to be monovalent, and for host-functionalized gold nanoparticles with FeTMPyP, the interaction was determined to be bivalent. Finally, the electrochemical properties of the electroactive supramolecular multivalent film were determined. (C) 2009 Elsevier B.V. All rights reserved ...

Page contains details about iota carrageenan-FeII supramolecular complexes . It has composition images, properties, Characterization methods, synthesis, applications and reference articles : nano.nature.com

The refined crystal structures of a proteolytic fragment of glucoamylase from Aspergillus awamori var. X100 have been determined at pH 6 and 4 to a resolution of 2.2 A and 2.4 A, respectively. The models include the equivalent of residues 1 to 471 of glucoamylase from Aspergillus niger and a complete interpretation of the solvent structure. The R-factors of the pH 6 and 4 structures are 0.14 and 0.12, respectively, with root-mean-square deviations of 0.014 A and 0.012 A from expected bondlengths. The enzyme has the general shape of a doughnut. The hole of the doughnut consists of a barrier of hydrophobic residues at the center, which separates two water-filled voids, one of which serves as the active site. Three clusters of water molecules extend laterally from the active site. One of the lateral clusters connects the deepest recess of the active site to the surface of the enzyme. The most significant difference in the pH 4 and 6 structures is the thermal parameter of water 500, the putative ...

TY - JOUR. T1 - New parameters controlling the effect of temperature on enzyme activity. AU - Daniel, R M. AU - Danson, M J. AU - Eisenthal, R. AU - Lee, C K. AU - Peterson, M E. N1 - ID number: ISI:000251875100038. PY - 2007. Y1 - 2007. N2 - Arising from careful measurements of the thermal behaviour of enzymes, a new model, the Equilibrium model, has been developed to explain more fully the effects of temperature on enzymes. The model describes the effect of temperature on enzyme activity in terms of a rapidly reversible active-inactive (but not denatured) transition, revealing an additional and reversible mechanism for enzyme activity loss in addition to irreversible thermal inactivation at high temperatures. Two new thermal parameters, T-eq and Delta H-eq, describe the active-inactive transition, and enable a complete description of the effect of temperature on enzyme activity. We describe here the Model and its fit to experimental data, methods for the determination of the Equilibrium model ...

The most simple method for solventless synthesis of supramolecular complex of CMCR·2BPY·BZP, [CMCR = C-methylcalix[4]resorcinarene, BPY = 4,4-bipyridine, BZP = benzophenone], is proposed. Although CMCR by itself is high melting point compound (above 300°C), CMCR was found to be dissolved in melt mixture of BPY and BZPeven below 120°C. In the mixture of the three components, the reaction occurs to form CMCR·2BPY·BZP supramolecular complex.

Steady natural convection heat transfer has been studied in porous wall, solar collector systems. The boundary conditions were: two isothermal walls at different temperatures, two horizontal bounding adiabatic walls, and either uniform or nonuniform heat generating porous layer without vents. The aspect ratio A was from 0.5 to 1.4. The Rayleigh number varied from 103 to 108 . The Darcy number was from 10−8 to 10−2 but the detailed studies were carried out only for 10−4 , an optimum value. The results are presented in terms of thermal parameters (θ, θmax , Nu) as a function of Ra and other nondimensional parameters (A=H/L, B=1/L, F=d/L, kr ). The isotherms and streamlines within the system are also produced. The overall results indicate that geometrical parameters are the most important parameters affecting the system performance.. ...

The occurrence of porosity during metal solidification is one of the major issues that impact the quality of castings. Quantitative information on the development of porosity is particularly important for safety critical components, such as automotive chassis parts and airframe primary structures. In this paper, we present two approaches to predict the location and volume fraction of porosity for aluminum alloy A356. In the first approach, the application of Neural Networks to predict porosity is examined. Results are compared with the established criteria functions and reported experimental findings. Neural Networks are shown to predict the occurrence of porosity with higher confidence than the existing thermal parameter based criteria functions. In the second approach, microporosity evolution is modeled mathematically. This model incorporates various solidification phenomena such as dendrite formation and growth, hydrogen evolution at the solid/liquid interface, solidification shrinkage, ...

Expression of FTCD () in thyroid gland tissue. Antibody staining with HPA020073, HPA030928, HPA030929 and HPA036114 in immunohistochemistry.

Khaw, L.E., Bohm, G.A., Metcalfe, S., Staunton, J. and Leadlay, P.F. (1998). „Mutational biosynthesis of novel rapamycins by a strain of Streptomyces hygroscopicus NRRL 5491 disrupted in rapL, encoding a putative lysine cyclodeaminase. J. Bacteriol. 180: 809-814. PMID 9473033 ...

We report the synthesis and characterization of novel mixed-metal binuclear ruthenium(ii)-cobalt(ii) photocatalysts for hydrogen evolution in acidic acetonitrile. First, 2-(2′-pyridyl)benzothiazole (pbt), 1, was reacted with RuCl3·xH2O to produce [Ru(pbt)2Cl2]·0.25CH3COCH3, 2, which was then reacted with 1,1 Dalton Discussion 13: Inorganic photophysics and photochemistry -Fundamentals and applications

International Lecture given by Professor Wolfgang Baumeister. In the past, macromolecular structures have been characterized by methods requiring their isolation and purification. When taken out of their functional context, information about their interactions within the cellular environment is lost. However, it is the interaction between the many molecular species inhabiting cells which underlies cellular functions. Many of them exist only fleetingly or, when integrated in supramolecular complexes, these may be rooted so deeply...

The Weak-Link Approach (WLA) is a supramolecular coordination-based assembly methodology, first introduced in 1998 by the Mirkin Group at Northwestern University. This method takes advantage of hemilabile ligands, containing both strong and weak binding moieties, that can coordinate to metal centers and quantitatively assemble into a single condensed closed structure (Figure 1). Unlike other supramolecular assembly methods, the WLA allows for the synthesis of supramolecular complexes that can be modulated from rigid closed structures to flexible open structures through reversible binding events that occur at the structural metal centers. The approach is general and has been applied to a variety of metal centers and ligand designs (Figure 2) including those with utility in catalysis and allosteric regulation. To date, Rh(I), Ir(I), Pd(II), Ru(II), Cu(I), Ni(II), and Pt(II) have been used as metal precursors in the WLA. The key component that enables in situ control of supramolecular ...

TY - JOUR. T1 - Aminoimidazole carboxamide ribonucleotide ameliorates experimental autoimmune uveitis. AU - Suzuki, Jun. AU - Yoshimura, Takeru. AU - Simeonova, Marina. AU - Takeuchi, Kimio. AU - Murakami, Yusuke. AU - Morizane, Yuki. AU - Miller, Joan W.. AU - Sobrin, Lucia. AU - Vavvas, Demetrios G.. PY - 2012/6/1. Y1 - 2012/6/1. N2 - PURPOSE. To investigate the anti-inflammatory effect of an adenosine monophosphate (AMP) analog, aminoimidazole carboxamide ribonucleotide (AICAR), in experimental autoimmune uveoretinitis (EAU). METHODS. C57BL/6 mice were injected daily with AICAR (200 mg/kg, intraperitoneally [IP]) from day 0, the day of interphotoreceptor retinoid-binding protein (IRBP) immunization, until day 21. The severity of uveitis was assessed clinically and histopathologically. T-cell proliferation and cytokine production of IFN-γ, IL-17, and IL-10 in response to IRBP stimulation were determined. In addition, regulatory T-cell (Treg) populations were measured. Co-stimulatory molecule ...

Looking for online definition of 5-aminoimidazole ribose 5-phosphate in the Medical Dictionary? 5-aminoimidazole ribose 5-phosphate explanation free. What is 5-aminoimidazole ribose 5-phosphate? Meaning of 5-aminoimidazole ribose 5-phosphate medical term. What does 5-aminoimidazole ribose 5-phosphate mean?

Christoph Cremer (born in Freiburg im Breisgau, Germany) is a German physicist and professor at the Ruprecht-Karls-University Heidelberg, honorary professor at the University of Mainz and group leader at the Institute of Molecular Biology (IMB) a newly established research centre on the campus of the Johannes Gutenberg University of Mainz, Germany, who has successfully overcome the conventional limit of resolution that applies to light based investigations (the Abbe limit) by a range of different methods (1971/1978 development of the concept of 4Pi-microscopy; 1996 localization microscopy SPDM; 1997 spatially structured illumination SMI). In September 2014 he founded the NPO LuciaOptics to support the use of Super-resolution microscopy in the fields of molecular biology, biomedicine, microbiology, virology, pharmaceutical sciences and diagnosis His actual microscope Vertico-SMI is the worlds fastest nano light microscope that allows large scale investigation of supramolecular complexes ...

Jorge Follana-Berná, Sairaman Seetharaman, Luis Martín-Gomis, Georgios Charalambidis, Adelais Trapali, Paul A. Karr, Athanassios G. Coutsolelos, Fernando Fernández-Lázaro, Francis DSouza and Ángela Sastre-Santos. Supramolecular Complex of a Fused Zinc Phthalocyanine-Zinc Porphyrin Dyad Assembled by Two Imidazole-C60 Units: Ultrafast Energy Transfer Followed by Electron Transfer. (2018) Physical Chemistry Chemical Physics 20, 7798-7807.. ...

We have investigated spectrally, crystallographically as well as computationally the charge transfer complexes involving newly synthesized N-aryl-N-methyl non-symmetric viologens (AMVs) and hexacyanoferrate(II) (HCF) anions. The supramolecular binding of AMVs and HCF was studied in solution and in the crystal state for one of the obtained complexes. Substituent effects on the electron affinities of the dicationic AMVs, determined using Mullikens theory [R. S. Mulliken, J. Am. Chem. Soc., 1952, 74, 811-824] were quantified. The structure of one of the AMV//Fe-II(CN)(6) pairs solved through Single-Crystal X-ray Diffraction (SCXRD), provided insights for the supramolecular binding of the anionic and cationic counterparts and the role of lattice water molecules. Supramolecular binding in solution, studied with the use of NMR spectroscopy, is in agreement with the results obtained in the solid state.. ...

Gap junctions are membrane specialization domains identified in most tissue types where cells abut each other. The connexin channels found in these membrane domains are conduits for direct cell-to-cell transfer of ions and molecules. Connexin43 (Cx43) is the most ubiquitous connexin, with critical roles in heart, skin, and brain. Several studies described the interaction between Cx43 and the cytoskeleton involving the actin binding proteins Zonula occludens (ZO-1) and drebrin, as well as with tubulin. However, a direct interaction has not been identified between drebrin and Cx43. In this study, co-IP and NMR experiments were used to demonstrate that the Cx43-CT directly interacts with the highly conserved N-terminus region of drebrin. Three Cx43-CT areas were found to be involved in drebrin binding, with residues 264-275 being critical for the interaction. Mimicking Src phosphorylation within this region (Y265) significantly disrupted the interaction between the Cx43-CT and drebrin. Immunofluorescence

Autophagy mediates lysosomal degradation of cytosolic components. Recent work has associated autophagic dysfunction with pathologies, including cancer and cardiovascular disease. To date, the identification of clinically-applicable drugs that modulate autophagy has been hampered by the lack of standardized assays capable of precisely reporting autophagic activity. We developed and implemented a high-content, flow-cytometry-based screening approach for rapid, precise, and quantitative measurements of pharmaceutical control over autophagy. Our assay allowed for time-resolved individual measurements of autolysosome formation and degradation, and endolysosomal activities under both basal and activated autophagy conditions. As proof of concept, we analyzed conventional autophagy regulators, including cardioprotective compounds aminoimidazole carboxamide ribonucleotide (AICAR), rapamycin, and resveratrol, and revealed striking conditional dependencies of rapamycin and autophagy inhibitor 3-methyladenine (3-MA

4.3.1.1 Aspartate ammonia-lyase 4.3.1.2 Methylaspartate ammonia-lyase 4.3.1.3 Histidine ammonia-lyase 4.3.1.4 Formimidoyltetrahydrofolate cyclodeaminase 4.3.1.5 Transferred entry: 4.3.1.23, 4.3.1.24 and 4.3.1.25 4.3.1.6 Beta-alanyl-CoA ammonia-lyase 4.3.1.7 Ethanolamine ammonia-lyase 4.3.1.8 Transferred entry: 2.5.1.61 4.3.1.9 Glucosaminate ammonia-lyase 4.3.1.10 Serine-sulfate ammonia-lyase 4.3.1.11 Deleted entry 4.3.1.12 Ornithine cyclodeaminase 4.3.1.13 Carbamoyl-serine ammonia-lyase 4.3.1.14 3-aminobutyryl-CoA ammonia-lyase 4.3.1.15 Diaminopropionate ammonia-lyase 4.3.1.16 Threo-3-hydroxy-L-aspartate ammonia-lyase 4.3.1.17 L-serine ammonia-lyase 4.3.1.18 D-serine ammonia-lyase 4.3.1.19 Threonine ammonia-lyase 4.3.1.20 Erythro-3-hydroxy-L-aspartate ammonia-lyase 4.3.1.21 Transferred entry: 4.3.1.9 4.3.1.22 3,4-dihydroxyphenylalanine reductive deaminase 4.3.1.23 Tyrosine ammonia-lyase 4.3.1.24 Phenylalanine ammonia-lyase 4.3.1.25 Phenylalanine/tyrosine ammonia-lyase 4.3.1.26 Transferred entry: ...

TY - JOUR. T1 - Shape- and functionality-controlled organization of TiO2- porphyrin-C60 assemblies for improved performance of photochemical solar cells. AU - Hasobe, Taku. AU - Fukuzumi, Shunichi. AU - Hattori, Shigeki. AU - Kamat, Prashant V.. PY - 2007/7/2. Y1 - 2007/7/2. N2 - Shape- and functionality-controlled organization of porphyrin derivatives-C60 supramolecular assemblies using TiO2 nanotubes and nanoparticles has been achieved for the development of photochemical solar cells. The differences in the efficiency of light-energy conversion of these solar cells are explained on the basis of the geometrical orientation of the porphyrins with respect to the TiO2 surface and the supramolecular complex formed with C60. The maximum photon-conversion efficiency (IPCE) of 60% obtained with TiO2 nanotube architecture is higher than the value obtained with nanoparticle architecture. The results presented in this study show the importance of substrate morphology in promoting electron transport ...

IDPs in their unbound state can transiently acquire secondary and tertiary structure. Describing such intrinsic structure is important to understand the transition between free and bound state, leading to supramolecular complexes with physiological interactors. IDP structure is highly dynamic and, therefore, difficult to study by conventional techniques. This work focuses on conformational analysis of the KID fragment of the Sic1 protein, an IDP with a key regulatory role in the cell-cycle of Saccharomyces cerevisiae. FT-IR spectroscopy, ESI-MS, and IM measurements are used to capture dynamic and short-lived conformational states, probing both secondary and tertiary protein structure. The results indicate that the isolated Sic1 KID retains dynamic helical structure and populates collapsed states of different compactness. A metastable, highly compact species is detected. Comparison between the fragment and the full-length protein suggests that chain length is crucial to the stabilization of ...

The latest results from the groups research to create a DNA targeting, light activated anticancer drug will be presented at the 231st American Chemical Society national meeting in Atlanta on March 26-30. Chemistry professor Karen J. Brewer reports that the group has developed supramolecular complexes that combine light-absorbing PDT agents and cisplatin like units. Previous anticancer molecules created by the group have contained platinum-based molecules that bind DNA. They have also developed new light activated systems able to photocleave DNA. This report combines these two approaches to target the drug to DNA using cisplatin like units, directing the light activation to tumor cells and the sub-cellular target, DNA. In the past, our light activated systems had to find the DNA within the cell, an often inefficient process. Now we have added the DNA targeting drug, Brewer said. We were working on cisplatin analogs before, so we have tied it to light activated systems. Cisplatin begins its ...

The cocrystal of nitrofurantoin with urea (C8H6N4O5)·(CH4N2O), a non-ionic supramolecular complex, has been studied. Nitrofurantoin (NF) is a widely used antibacterial drug for the oral treatment of infections of the urinary tract. Characterization of the cocrystal of nitrofurantoin with urea (NF-urea) was p

Guest Book for Meningitis Outbreak Victims of Columbus, GA. Share your condolences, send flowers, find funeral home information. Guestbook provided by Columbus Ledger-Enquirer.

I am an Aesthetic & Reconstructive Plastic Surgeon who has been in practice for 25 years now. I am currently the President of the Phil. Academy for Head & Neck Surgery.