Search results for "PCD"
showing 10 items of 20 documents
Multi-isotopic and trace element evidence against different formation pathways for oyster microstructures
2021
Geochimica et cosmochimica acta 308, 326-352 (2021). doi:10.1016/j.gca.2021.06.012
Towards controlling PCDD/F production in a multi-fuel fired BFB boiler using two sulfur addition strategies. Part II: Thermodynamic analysis
2014
Abstract A staged equilibrium process model was developed for a bubbling fluidized bed boiler firing SRF, bark and sludge. The model was used to study the influence of sulfur addition strategies (S-pellet additive and peat co-firing) on the behavior of copper, bromine, and alkalis. Aerosol samples collected from the backpass of the boiler were used to validate the chemistry predicted by the model. The model revealed that Cu existed as Cu 2 S (s3) in the reducing zone, and CuCl (g) (for all test cases) and CuO (s) (during peat co-firing) in the oxidation zones. CuBr 3(g) was also present after the introduction of tertiary air. However the model failed to predict the formation of CuSO 4 , an …
Apoptosis: focus on sea urchin development
2009
It has been proposed that the apoptosis is an essential requirement for the evolution of all animals, in fact the apoptotic program is highly conserved from nematodes to mammals. Throughout development, apoptosis is employed by multicellular organisms to eliminate damaged or unnecessary cells. Here, we will discuss both developmental programmed cell death (PCD) under normal conditions and stress induced apoptosis, in sea urchin embryos. Sea urchin represent an excellent model system for studying embryogenesis and cellular processes involved in metamorphosis. PCD plays an essential role in sculpting and remodelling the embryos and larvae undergoing metamorphosis. Moreover, this marine organi…
Cadherin 23 is a component of the transient lateral links in the developing hair bundles of cochlear sensory cells
2005
AbstractCadherin 23 is required for normal development of the sensory hair bundle, and recent evidence suggests it is a component of the tip links, filamentous structures thought to gate the hair cells' mechano-electrical transducer channels. Antibodies against unique peptide epitopes were used to study the properties of cadherin 23 and its spatio-temporal expression patterns in developing cochlear hair cells. In the rat, intra- and extracellular domain epitopes are readily detected in the developing hair bundle between E18 and P5, and become progressively restricted to the distal tip of the hair bundle. From P13 onwards, these epitopes are no longer detected in hair bundles, but immunoreac…
Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle
2002
Deaf-blindness in three distinct genetic forms of Usher type I syndrome (USH1) is caused by defects in myosin VIIa, harmonin and cadherin 23. Despite being critical for hearing, the functions of these proteins in the inner ear remain elusive. Here we show that harmonin, a PDZ domain-containing protein, and cadherin 23 are both present in the growing stereocilia and that they bind to each other. Moreover, we demonstrate that harmonin b is an F-actin-bundling protein, which is thus likely to anchor cadherin 23 to the stereocilia microfilaments, thereby identifying a novel anchorage mode of the cadherins to the actin cytoskeleton. Moreover, harmonin b interacts directly with myosin VIIa, and i…
Towards controlling PCDD/F production in a multi-fuel fired BFB boiler using two sulfur addition strategies. Part I: Experimental campaign and results
2014
Abstract Levels of PCDD/F production in a 140 MW th bubbling fluidized bed boiler were measured. The boiler uses solid recovered fuel, bark and sludge. Homologue distribution patterns suggest the de novo mechanism is the main pathway for the generation of dioxin and furans in the post combustion zones of the boiler. Two modes of sulfur addition were tested to induce the deactivation of Cu which has been identified as the prime catalyst of this mechanism. First, S-pellet promoted Cu sulfation as supported by aerosol sampling data and resulted in a decrease in PCDD/F levels. The second approach was adding sulfur through peat; this resulted in an increase in PCDD/F concentration. Factors such …
The Cellular Function of the Usher Gene Product Myosin VIIa is Specified by Its Ligands
2003
Defects in myosin Vlla are responsible for Usher Syndrome 1B (Weil et al., 1995). Human Usher syndrome (USH), named after the British ophthalmologist Charles Usher (Usher, 1914), is the most common hereditary form of combined blind-and deafness (~ 50% of cases in the developed countries). USH designates a group of clinically and genetically heterogeneous disorders with hearing loss and retinitis pigmentosa (RP). Three different USH types (USH1, 2 and 3; see Table 1) can be distinguished according to the degree of clinical symptomes. USH1 is the most severe subtype, characterized by severe to profound congenital sensorineuronal deafness, constant vestibular dysfunction (balance deficiency) a…
Genome-wide association analysis on normal hearing function identifies PCDH20 and SLC28A3 as candidates for hearing function and loss.
2015
Hearing loss and individual differences in normal hearing both have a substantial genetic basis. Although many new genes contributing to deafness have been identified, very little is known about genes/variants modulating the normal range of hearing ability. To fill this gap, we performed a two-stage meta-analysis on hearing thresholds (tested at 0.25, 0.5, 1, 2, 4, 8 kHz) and on pure-tone averages (low-, medium-and high-frequency thresholds grouped) in several isolated populations from Italy and Central Asia (total N = 2636). Here, we detected two genome-wide significant loci close to PCDH20 and SLC28A3 (top hits: rs78043697, P = 4.71E-10 and rs7032430, P = 2.39E-09, respectively). For both…
Cancer Risk Near a Polluted River in Finland
2004
The River Kymijoki in southern Finland is heavily polluted with polychlorinated dibenzo-p-dioxins and dibenzofurans and may pose a health threat to local residents, especially farmers. In this study we investigated cancer risk in people living near the river (less than 20.0 km) in 1980. We used a geographic information system, which stores registry data, in 500 m times 500 m grid squares, from the Population Register Centre, Statistics Finland, and Finnish Cancer Registry. From 1981 to 2000, cancer incidence in all people (N = 188884) and in farmers (n = 11132) residing in the study area was at the level expected based on national rates. Relative risks for total cancer and 27 cancer subtype…
Immunofluorescence Analysis as a Diagnostic Tool in a Spanish Cohort of Patients with Suspected Primary Ciliary Dyskinesia
2020
Primary ciliary dyskinesia (PCD) is an autosomal recessive rare disease caused by an alteration of ciliary structure. Immunofluorescence, consisting in the detection of the presence and distribution of cilia proteins in human respiratory cells by fluorescence, has been recently proposed as a technique to improve understanding of disease-causing genes and diagnosis rate in PCD. The objective of this study is to determine the accuracy of a panel of four fluorescently labeled antibodies (DNAH5, DNALI1, GAS8 and RSPH4A or RSPH9) as a PCD diagnostic tool in the absence of transmission electron microscopy analysis. The panel was tested in nasal brushing samples of 74 patients with clinical suspic…