Search results for " complex"
showing 10 items of 3391 documents
CNGB3 mutations account for 50% of all cases with autosomal recessive achromatopsia
2005
Contains fulltext : 47591.pdf (Publisher’s version ) (Closed access) Achromatopsia is a congenital, autosomal recessively inherited disorder characterized by a lack of color discrimination, low visual acuity (<0.2), photophobia, and nystagmus. Mutations in the genes for CNGA3, CNGB3, and GNAT2 have been associated with this disorder. Here, we analyzed the spectrum and prevalence of CNGB3 gene mutations in a cohort of 341 independent patients with achromatopsia. In 163 patients, CNGB3 mutations could be identified. A total of 105 achromats carried apparent homozygous mutations, 44 were compound (double) heterozygotes, and 14 patients had only a single mutant allele. The derived CNGB3 mutatio…
Geochemistry of REE, Zr and HF in a wide range of pH and water composition: The Nevado del Ruiz volcano hydrothermal system (Colombia)
2015
International audience; The geochemical behaviour of Rare Earth Elements, Zr and Hf was investigated in the thermal waters of Nevado del Ruiz volcano system. A wide range of pH, between 1.0 and 8.8, characterizes these fluids. The acidic waters are sulphate dominated with different Cl/SO4 ratios. The important role of the pH and the ionic complexes for the distribution of REE, Zr and Hf in the aqueous phase was evidenced. The pH rules the precipitation of authigenic Fe and Al oxyhydroxides producing changes in REE, Zr, Hf amounts and strong anomalies of Cerium. The precipitation of alunite and jarosite removes LREE from the solution, changing the REE distribution in acidic waters.Y–Ho and Z…
Polycarboxylic acids in sea water: acid–base properties, solubilities, activity coefficients, and complex formation constants at different salinities
2016
This paper reports the results of the investigations carried out in synthetic sea water at different salinities for different classes of polycarboxylic acids. The investigations can be summarized as follows: (a) Determination of the protonation constants in such multicomponent solution in a salinity range 15 ≤ S ≤ 45, at t = 25 °C, for the linear dicarboxylic acids HOOC-(CH2) n –COOH (0 ≤ n ≤ 8), and aromatic polycarboxylic acids (o-phthalic and 1,2,4-benzenetricarboxylic acids). For malonic, succinic, 1,2,3-benzenetricarboxylic, and 1,2,3,4-benzenetetracarboxylic acids, investigations were also carried out at t = 10 and 37 °C; (b) Determination of the total and intrinsic solubility (S T an…
Solubility and acid-base properties and activity coefficients of chitosan in different ionic media and at different ionic strengths, at T=25°C
2009
Studies on the acid-base properties and solubility of a polyammonium polyelectrolyte (chitosan) with different molecular weights (MW 310 and 50 kDa), were performed at T=25 °C, in the pH range 2.5–7. The protonation of chitosan was investigated by potentiometry ([H+]-glass electrode) in NaCl, NaNO3 and mixed NaNO3+Na2SO4 ionic media, at different ionic strengths. Protonation constants were calculated as a function of dissociation degree α by means of two different models, namely, a simple linear model and the modified Henderson–Hasselbalch equation. Experimental data were also fitted using a model independent of α (Diprotic-like model), according to which the acid-base properties can be sim…
Targeting Acute Leukemia and Cancer by High-Affinity T-Cell-Receptor Transfer
2003
Accumulation and subsequent overexpression of human mdm2 (hdm2) and altered p53 protein is associated with high-level presentation of hdm2 and wild-type (wt) p53 derived peptides by major histocompatibility complex (MHC) class I molecules on a wide range of malignant cells. A major barrier to the design of broad-spectrum hdm2 and p53 specific immunotherapeutics for leukemia and cancer, however, has been the observation that low-level expression of hdm2 and wt p53 peptides by non-transformed tissues and cells results in self-tolerance of T-lymphocytes with high avidity for self-class I MHC / self-peptide complexes. Although the peripheral T-cell repertoire is mostly devoid of such high-avidi…
F1-ATPase from Micrococcus sp. ATCC 398. Purification by Ion-Exchange Chromatography and Further Characterization. (Auto)proteolysis and Dissociative…
1977
The preparation of highly purified F1-ATPase from Micrococcus sp. ATCC 398 by application of DEAE-Sepharose CL-6B chromatography as final step is described. This enzyme consists of five subunits of different molecular weight: alpha (65000), beta (55000),gamma (35000), delta (20000), and epsilon (17000). Disc electrophoresis on 5% polyacrylamide gels removes the epsilon-polypeptide yielding an active ATPase complex with four different subunits: alpha, beta, gamma, delta. Additionally, by variation of the ionic strength delta can (partly) removed allowing the isolation by disc electrophoresis of an active ATPase complex which consists only of three different subunits alpha, beta, and gamma. I…
m6A RNA methylation of major satellite repeat transcripts facilitates chromatin association and RNA:DNA hybrid formation in mouse heterochromatin
2021
Abstract Heterochromatin has essential functions in maintaining chromosome structure, in protecting genome integrity and in stabilizing gene expression programs. Heterochromatin is often nucleated by underlying DNA repeat sequences, such as major satellite repeats (MSR) and long interspersed nuclear elements (LINE). In order to establish heterochromatin, MSR and LINE elements need to be transcriptionally competent and generate non-coding repeat RNA that remain chromatin associated. We explored whether these heterochromatic RNA, similar to DNA and histones, may be methylated, particularly for 5-methylcytosine (5mC) or methyl-6-adenosine (m6A). Our analysis in mouse ES cells identifies only b…
3D in Suspension versus 2D in Adhesion: molecular profiles in stemness and mesenchymal differentiation of Spheroids from Adipose-derived Stem Cells
2021
Purpose: Adipose stem cells (ASCs) represent a reliable source of stem cells with a widely demonstrated potential in regenerative medicine and tissue engineering applications. New recent insights suggest that three-dimensional (3D) models may closely mimic the native tissue properties; spheroids from adipose derived stem cells (S-ASCs) exhibit enhanced regenerative abilities compared with those of 2D models. Stem cell therapy success is determined by “cell-quality”; for this reason, microRNA profiles, the involvement of stress signals and cellular aging need to be further investigated. Material and Methods: Adipose tissue was collected from healthy individuals, 44 females and 17 males, afte…
Evaluation of the pulse wave arrival time as a marker for blood pressure changes in critically ill infants and children
1995
Objective. Pulse arrival time (PAT), which is the interval between the R wave of the electrocardiogram (ECG) and the arrival of the pulse wave peripherally, has been reported to be suitable for use as an indirect measure of blood pressure change. The purpose of this study was to evaluate, in critically ill infants and children, the degree to which 1/PAT covaries with systolic, diastolic, and mean blood pressure, as well as heart rate.Methods. A laboratory device was used to calculate PAT in real time from the ECG and the plethysmographic curve of pulse oximetry used for routine monitoring. Calculated PAT and corresponding blood pressures and heart rate were stored on hard disk. A total of 1…
Adolescents en conflicte amb la llei: víctimes o victimàries?: una aproximació des de la sociologia i el treball social
2008
En éste artículo se realiza un aproximación al fenómeno de la delincuencia juvenil, desde el paradigma de la vulnerabilidad y la exclusión social. Desde ésta aproximación se realiza la investigación en un territorio concreto, como es el municipio de Burjassot en el área metropolitana de València (País Valencià), se profundiza en las variables de vulnerabilidad de éstos adolescentes, lo que nos permite relacionar su realidad con la nueva cuestión social. El trabajo incide en la complejidad del fenómeno, huyendo de explicaciones lineales y sencillas, extrayendo como variables fundamentales a investigar: la realidad educativa, el barrio de residencia en el municipio, la existencia previa de in…