The dentin sialophosphoprotein (DSPP) gene (4q21.3) encodes two major noncollagenous dentin matrix proteins: dentin sialoprotein (DSP) and dentin phosphoprotein (DPP). Defects in the human gene ...encoding DSPP cause inherited dentin defects, and these defects can be associated with bilateral progressive high-frequency sensorineural hearing loss. Clinically, five different patterns of inherited dentin defects are distinguished and are classified as dentinogenesis imperfecta (DGI) types I, II, and III, and dentin dysplasia types I and II. The genetic basis for this clinical heterogeneity is unknown. Among the 11 members recruited from the studied kindred, five were affected with autosomal dominant DGI type II. The mutation (g.1188C-->G, IVS2-3C-->G) lay in the third from the last nucleotide of intron 2 and changed its sequence from CAG to GAG. The mutation was correlated with the affection status and was absent in 104 unaffected individuals (208 alleles) with the same ethnic and geological background. The proband was in the primary dentition stage and presented with multiple pulp exposures. The occlusal surface of his dental enamel was generally abraded, and the dentin was heavily worn and uniformly shaded brown. The dental pulp chambers appeared originally to be within normal limits without any sign of obliteration, but over time (by age 4), the pulp chambers became partially or completely obliterated. The oldest affected member (age 59) showed mild hearing loss at high-frequency (8 kHz). Permanent dentition was severely affected in the adults, who had advanced dental attrition, premature loss of teeth, and extensive dental reconstruction.
Can kindreds with tooth agenesis caused by MSX1 or PAX9 mutations be distinguished by their phenotypes? We have identified an MSX1second bicuspids and mandibular central incisors. The dominant ...phenotype is apparently due to haploinsufficiency. We analyzed patterns of partial tooth agenesis in seven kindreds with defined MSX1 mutations and ten kindreds with defined PAX9 mutations. The probability of missing a particular type of tooth is always bilaterally symmetrical, but differences exist between the maxilla and mandible. MSX1-associated oligodontia typically includes missing maxillary and mandibular second bicuspids and maxillary first bicuspids. The most distinguishing feature of MSX1-associated oligodontia is the frequent (75%) absence of maxillary first bicuspids, while the most distinguishing feature of PAX9-associated oligodontia is the frequent (> 80%) absence of the maxillary and mandibular second molars.
Background
A high prevalence of cholestatic disease, including gallbladder mucocele (GBM), has been reported in dogs with naturally occurring pituitary‐dependent hyperadrenocorticism (PDH).
...Hypothesis/Objectives
Differences exist in the clinical features of dogs with PDH and concurrent cholestatic disease, and also is the management of these dogs with trilostane.
Animals
Sixty‐five client‐owned dogs with naturally occurring PDH.
Methods
This was a retrospective, observational case series. Each dog was treated with trilostane for at least 3 months before the study, and had a good clinical response, as determined by owners. Statistical comparisons of clinical signs, results of routine blood tests, basal and post‐ACTH cortisol concentration, and optimal trilostane dosage were made after dogs were separated into the following 3 groups by ultrasonographic imaging: normal on ultrasound (NOU) group, cholestasis group, and GBM group.
Results
The GBM group had more severe clinical signs and significantly different total serum cholesterol concentration and post‐ACTH stimulation cortisol concentration at the time of diagnosis. Dogs that weighed <6 kg had a significantly higher prevalence of cholestatic disease than did the other dogs (P = .003). The optimal trilostane dosages for the GBM and cholestasis groups were 2.5 and 1.5 times the dosage of the NOU group, respectively (P < .001).
Conclusions and Clinical Importance
Gallbladder disease associated with cholestatic disease is correlated with PDH in dogs, in both its clinical features and drug management. These findings may be associated with hypercholesterolemia, unidentified genetic factors, and the hydrophobic nature of trilostane.
Objective: This study was designed to prospectively investigate the efficacy and cognitive adverse effects of stereotactic bilateral anterior cingulotomy as a treatment for refractory ...obsessive–compulsive (OCD) patients for 12 months.
Method: Patients were eligible if they had severe OCD and rigorous treatments had been unsuccessful. Yale‐Brown Obsessive–Compulsive Scale (Y‐BOCS), Clinical Global Impression (CGI) and neuropsychological tests were used to assess the efficacy and cognitive changes of cingulotomy before and 12 months after operation.
Results: The mean improvement rate of the Y‐BOCS scores achieved from baseline was 36.0%. Out of 14 patients six met responder criteria; 35% or higher improvement rate on Y‐BOCS and CGI improvement of very much or much better at 12‐month follow‐up. There was no significant cognitive dysfunction after cingulotomy.
Conclusion: Anterior cingulotomy shows few cognitive adverse effects, with about half of the OCD patients demonstrating significant symptomatic improvement.
•A new hybrid energy harvester design has been proposed and verified.•Low frequency driven electromagnetic-triboelectric hybrid mechanism.•Halbach array converges the magnetic flux density and helps ...higher power density.•Sandpaper assisted inexpensive micro-patterned PDMS film has been utilized.
We have proposed a new design of hybridized electromagnetic-triboelectric energy harvester using Halbach magnet array from handshaking vibration and validated it theoretically and experimentally. The Halbach array helps to enhance the magnetic flux density and reduce the overall volume as well as generate high power at low frequency. In particular, the proposed dual Halbach array allows the concentrated magnetic flux lines to interact with the same coil in a way where maximum flux linkage occurs. To obtain much higher power generation in low amplitude and low frequency vibrations, the proposed harvester was comprised of a Halbach magnet array, sandpaper passed microstructure PDMS, TENG, and magnetic springs. A prototype of the hybridized energy harvester has been fabricated and tested both using a vibration exciter test and by manual handshaking. Under vibration exciter test, the fabricated prototype of hybridized harvester delivered a high output current and power of 2.9mA and 11.75mW, respectively, corresponding to a volume power density of 381W/m3 under a loading resistance of 1.39kΩ at 5Hz resonant frequency and 0.5g acceleration. It is also capable of delivering output current and power of 2.85mA and 8.1mW, respectively, by handshaking vibration. The fabricated hybridized harvester exhibited much higher power density than the recently reported similar works. Our proposed work takes a significant step toward hybrid energy harvesting from human-body-induced motions such as handshaking, walking, running and its potential applications in self powered portable electronics.
We report direct measurements of electrical transport through poly(dA)-poly(dT) and poly(dG)-poly(dC) DNA molecules containing identical base pairs. The observed experimental results suggest that ...electrical transport through DNA molecules occurs by polaron hopping. We have also investigated the effect of gate voltage on the current-voltage curve. It demonstrates the possibility of a DNA field-effect transistor operating at room temperature. Moreover, the gate-voltage dependent transport measurements show that poly(dA)-poly(dT) behaves as an n-type semiconductor, whereas poly(dG)-poly(dC) behaves as a p-type semiconductor.
The enhancement of magnetocaloric effect (MCE) and the extension of effective magnetic refrigeration temperature region near room temperature are critical requirements for highly efficient and ...environment friendly cooling technologies. The large isothermal entropy change of the first-order phase transition material and broader temperature range of the second-order phase transition material are combined into the composite form of La(Fe,Mn,Si)13-H and Gd with their transition temperatures close to room temperature. In this work, wire composites were fabricated using the La (Fe,Mn,Si)13-H powders filled inside the Gd tube. The magnetocaloric properties of the wire composite are convoluted from individual constituents. The dual transitions temperature were observed at 287 K and 292 K. The isothermal entropy change response of the wire composite can be obtained by using the simulation with the combined weight of the Gd tube and La (Fe,Mn,Si)13-H powders. The maximum isothermal entropy change (−ΔSiso) at μoH= 5 T calculated from isothermal magnetization measurements are 8.7 for Gd tube, 16.9 for La(Fe,Mn,Si)13-H powders, and 10.2 J/kg•K for a wire composite. The magnetocaloric properties including the order of phase transition of the wire composite are close to second-order type due to the higher weight fraction of Gd tube.
●Gd tube and La(Fe,Mn,Si)13-H alloy powders are used to fabricate into the form of the wire composite.●Gd tube itself is a good magnetocaloric material and can act as a protective layer for the MCE powders inside.●Wire composite shows the benefits of combing the effect of FOPT and SOPT.
The grain size effect on the recovery strain and recovery stress was systematically examined over a very wide grain-size range (between 1.4 μm and 171 μm) in the Fe-15.9Mn-5.1Si-8.1Cr-3.9Ni-0.02C ...alloy for a better understanding of the grain size effect on shape memory properties of the Fe–Mn–Si based alloys. The recovery strain rapidly increases with grain size up to approximately 16 μm and then gradually increases with a further increase in grain size. A linear relationship between recovery strain and logd (d: grain size) is observed in the whole grain size range. The densities of grain boundaries and annealing twin boundaries also exhibit the linear relationship with logd, but the trend is opposite, suggesting that recovery strain is directly related to the densities of grain boundaries and twin boundaries and the former increases as the latter decreases. The recovery stress behavior during heating and cooling of the samples is different depending on the grain size range. When the grain size is smaller than 6 μm, yielding does not occur during sample cooling. When the grain size is in the range between 16 μm and 33 μm, yielding occurs during cooling. When the grain size is larger than 46 μm, yielding and forward martensitic transformation -occur in sequence during cooling. The highest recovery stress is obtained at a grain size of 6 μm, which is the largest grain size where yielding can be avoided during cooling, and the lowest recovery stress is obtained at a grain size of 171 μm, where both yielding and forward martensitic transformation occur during cooling. Depending on the degree of grain refinement, the quality of improvement in recovery stress and recovery strain is expected to be different. Simultaneous improvement of recovery stress and strain is predicted to be hard to effectively achieve only through grain refinement.
Aims.
We study ten molecular transitions obtained from an unbiased 3 mm molecular line survey using the IRAM 30 m telescope toward 409 compact dust clumps identified by the APEX Telescope Large Area ...Survey of the Galaxy (ATLASGAL) to better understand the photodissociation regions (PDRs) associated with these clumps. The main goal of this study is to investigate whether the abundances of the selected molecules show any variations resulting from the PDR chemistry in different clump environments.
Methods.
We selected HCO, HOC
+
, C
2
H, c-C
3
H
2
, CN, H
13
CN, HC
15
N, and HN
13
C as PDR tracers, and H
13
CO
+
and C
18
O as dense gas tracers. By using estimated optical depths of C
2
H and H
13
CN and assuming optically thin emission for other molecular transitions, we derived the column densities of those molecules and their abundances. To assess the influence of the presence and strength of ultra-violet radiation, we compare abundances of three groups of the clumps: H
II
regions, infrared bright non-H
II
regions, and infrared dark non-H
II
regions.
Results.
We detected C
18
O, H
13
CO
+
, C
2
H, c-C
3
H
2
, CN, and HN
13
C toward most of the observed dust clumps (detection rate >94%), and H
13
CN is also detected with a detection rate of 75%. On the other hand, HCO and HC
15
N show detection rates of 32 and 39%, respectively, toward the clumps, which are mostly associated with H
II
region sources: detection rates of HCO and HC
15
N toward the H
II
regions are 66 and 79%. We find that the abundances of HCO, CN, C
2
H, and c-C
3
H
2
decrease as the H
2
column density increases, indicating high visual extinction, while those of high-density tracers (i.e., H
13
CO
+
and HC
15
N) are constant. In addition,
N
(HCO)/
N
(H
13
CO
+
) ratios significantly decrease as H
2
column density increases, and, in particular, 82 clumps have
X
(HCO) ≳ 10
−10
and
N
(HCO)/
N
(H
13
CO
+
) ≳ 1, which are indications of far-ultraviolet (FUV) chemistry. This suggests the observed HCO abundances are likely associated with FUV radiation illuminating the PDRs. We also find that high
N
(c-C
3
H
2
)/
N
(C
2
H) ratios found for H
II
regions that have high HCO abundances (≳10
−10
) are associated with more evolved clumps with high
L
bol
/
M
clump
. This trend might be associated with grain-surface processes, which determine the initial abundances of these molecules, and time-dependent effects in the clumps corresponding to the envelopes around dense PDRs and H
II
regions. In addition, some fraction of the measured abundances of the small hydrocarbons of the H
II
sources may be the result of the photodissociation of PAH molecules.
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