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Egg size is a fast-evolving trait among Drosophilids expected to change the spatial distribution of morphogens that pattern the embryonic axes. Here we asked whether the patterning of the dorsal region of the embryo by the Decapentaplegic/Bone Morphogenetic Protein-4 (DPP/BMP-4) gradient is scaled among Drosophila species with different egg sizes. This region specifies the extra-embryonic tissue amnioserosa and the ectoderm. We find that the entire dorsal region scales with embryo size, but the gene expression patterns regulated by DPP are not proportional, suggesting that the DPP gradient is differentially scaled during evolution. To further test whether the DPP gradient can scale or not in Drosophila melanogaster, we created embryos with expanded dorsal regions that mimic changes in scale seen in other species and measured the resulting domains of DPP-target genes. We find that the proportions of these domains are not maintained, suggesting that the DPP gradient is unable to scale in the embryo. These and previous findings suggest that the embryonic dorso-ventral patterning lack scaling in the ventral and dorsal sides but is robust in the lateral region where the neuroectoderm is specified and two opposing gradients, Dorsal/NFkappa-B and DPP, intersect. We propose that the lack of scaling of the DPP gradient may contribute to changes in the size of the amnioserosa and the numbers of ectodermal cells with specific cortical tensions, which are expected to generate distinct mechanical forces for gastrulating embryos of different sizes.

The effect of temperature on the production of aromatic compounds using Heisei Miyazaki yeast MF062 was compared between 10 industrial yeasts. All yeasts tested produced characteristic patterns of alcohols and esters in fermentation tests with rice-koji at 20, 28 and 38°C. The concentration and composition in mature moromi with rice-koji at 20, 28 and 38°C were almost the same as those with barley-koji. Therefore, it was suggested that fermentation temperature is an important factor in the production of aromatic compounds. MF062 produced almost the same concentration of β-phenethyl alcohol at both 38 and 28°C. The concentration was higher than that generated by the other 10 yeasts. MF062 produced higher concentrations of i-butyl alcohol than the other yeasts at higher fermentation temperatures. Moreover, compared with the other yeasts, MF062 produced a lower concentration of acetate, which can give an off-flavour in excess concentrations in shochu. The production of acetoin was divided into two groups – a high producing group and a low producing group – at all temperatures. MF062 belonged to the latter group and showed preferred characteristics in the production of shochu, resulting in a high concentration of preferred aromatic compounds and a low concentration of compounds that impart an off-flavour. Copyright © 2013 The Institute of Brewing & Distilling

OBJECTIVE: Although resection of isolated pulmonary metastases is a common treatment in other primaries such as colon cancer, the role of lung metastasectomy of breast cancer is still unclear. The objective of the present study was to investigate the clinical outcome of our operated patients with isolated pulmonary breast cancer metastases and discuss the clinical implication of resection with curative intent for them. METHODS: We retrospectively analyzed 86 female patients with histologically proven isolated pulmonary metastases from breast cancer who were treated with surgery of curative intent between January 1980 and September 2010 at 7 Japanese hospitals. The mean age of them was 50 years, the median disease free interval (DFI) from the definitive surgery for the primary breast cancer was 4.6 years (range: 0.5−20.4 years) and the median follow-up after lung metastasectomy was 4.1 years (range: 0.3−30.9 years). RESULTS: Lung metastasectomy was performed by thoracotomy in 22 patients and by video-assisted thoracoscopic surgical resection in 64 patients. The number of metastatic foci resected was one for 79 patients, two for 6, and unknown for 1. Lymph node resection was performed for 12 patients, was not done for 69, and unknown for 5. Five-year survival rates after lung metastasectomy were 68% in the patients. The main prognostic factor was the DFI. Five-year survival rates for patients with DFI of longer than 2 years (n = 72) and those with DFI of shorter than 2 years (n=14) were 74.5% and 33.8%, respectively (p = 0.0006). Five-year survival rates after lung metastasectomy were 68% in patients without macroscopic residual pulmonary tumor after lung metastasectomy (n = 69) and 34% in those with macroscopic residual tumor (n = 12) (p = 0.023). Five-year disease free survival after lung metastasectomy for patients without macroscopic residual tumor was 51 %. Five-year survival rates were 78% in patient with endocrine hormonal therapy following lung metastasectomy (n = 51) and 53% in those without endocrine hormonal therapy (n = 35) (p = 0.045). No prognostic influence could be demonstrated for age, number of initial axillary node metastases, primary tumor size. CONCLUSION: The prognosis of the breast cancer patients with isolated pulmonary metastases who underwent metastasectomy with curative intent was good. It was affected by the biological aspects of the primary tumor, “curativity” of the resection, and systemic treatments after lung resection. It may be expected that some of those patients will be cured by surgery with curative intent. Citation Information: Cancer Res 2011;71(24 Suppl):Abstract nr P2-15-04.

Seven yeasts with superior ability in terms of alcohol fermentation and aromatic ingredient generation were isolated from 272 wild yeasts obtained from shochu mashes of shochu breweries. These seven yeasts were examined for their fermentation ability with rice and sweet potato using small scale of fermentation tests. Moreover, their thermotolerance was evaluated by growth tests and fermentation tests with barley koji. Among the isolated seven yeasts, the shochu yeast “MF062” was superior in the characteristics of fast fermentation, high alcohol production, aromatic ingredient generation and thermotolerance. It was named the “Heisei Miyazaki Yeast”. From the results of analyses of physiological and genetic characteristics of MF062, it was shown to be a Saccharomyces cerevisiae, but it showed different characteristics from the industrial yeasts used in shochu production. Using a sweet potato shochu brewing test, with 60 kg of raw materials and yeast MF062, it was confirmed that a high alcohol production yield and a high quality shochu could be obtained.

Effects of anaesthesia on serum concentrations of thyroid hormones during and soon after abdominal surgery were examined in 29 patients undergoing cholecystectomy (n = 22) or removal of gastric cancer (n = 7). They were given one of the following anaesthetics in combination with nitrous oxide in oxygen: epidural bupivacaine, enflurane, pentazocine, ketamine, halothane, epidural bupivacaine and enflurane. Regardless of type of anaesthesia, T3 decreased significantly during and after surgery. T4 and rT3 increased markedly when either enflurane or halothane was given but not with the other anaesthetic agents; they then decreased toward pre-surgical levels after surgery. There was no correlation between changes in rT3 and those in cortisol or free fatty acids. TSH fluctuated little. These results show that the increases in rT3 and T4 during and soon after surgery are due not to surgical trauma but to inhalational anaesthetics such as enflurane and halothane.

Summary Using a highly sensitive radioimmunoassay (RIA) of estramustine binding protein (EMBP) with the antibody raised against rat-EMBP, the concentration of EMBP in human benign prostatic hypertrophy (BPH) tissue was evaluated. Using mechanical separation procedure, EMBP was found significantly higher in the epithelium (10.7 ng/mg protein) than in the stroma (2.9 ng/mg protein). When human BPH was classified into glandular, fibromuscular and mixed type, the concentration of EMBP was the highest in glandular type (15.7 ng/mg protein) and the lowest in fibromuscular type (10.8 ng/mg protein). Zusammenfassung Unter Verwendung eines hoch empfindlichen Radioimmunassays fur Oestramustin-Bindungs-Protein (EMBP) mit einem Antikorper gegen Ratten-EMBP wurde die Konzentration von EMBP im Prostatagewebe bei benigner Hypertrophic bestimmt. Das Gewebe wurde mechanisch getrennt; EMBP wurde signifikant hoher im Epithel (10,7 ng/mg Protein) als im Stroma (2,9 ng/mg Protein) gefunden. Wenn menschliche benigne Prostatahypertrophie eingeteilt war in glandulare, fibromuskulare und gemischte Typen, dann ergab sich die hochste Konzentration fur EMBP (15,7 ng/mg Protein) im glandularen Typ und die geringste im fibromuskularen Typ (10,8 ng/mg Protein).

(2008). Fatigue properties of laser weld in aluminium alloy. Welding International: Vol. 22, No. 10, pp. 705-711.

We have investigated a polycrystalline silicon (poly-Si)/chemical vapor deposited titanium nitride (CVD-TiN) stacked structure as a metal gate with a high-k for p-type metal insulator semiconductor field effect transistors (p-MISFETs). A divided-CVD method provided an appropriate effective work function (4.9–5.2 eV) on HfSiON for p-MISFETs. However, the deposition of poly-Si on CVD-TiN films shifted the effective work function to a midgap (~4.6 eV), and Ti, Hf, and Si diffused into poly-Si/CVD-TiN/high-k structures during poly-Si deposition. Then, we found that an increase in the deposition temperature of CVD-TiN films and the insertion of a physical vapor deposited (PVD)-TiN film between the poly-Si and CVD-TiN layers are effective in suppressing these diffusions. In particular, the insertion of the PVD-TiN film provided an appropriate effective work function of 4.9 eV. Therefore, we found that the diffusion control techniques for poly-Si/TiN/high-k stacked structures are highly effective for obtaining the appropriate work function for p-MISFETs.

HfxSiyO1−x−y layers with thicknesses of 2 and 10 nm were measured by medium energy ion scattering (MEIS) with a toroidal electrostatic analyzer (TEA), in which the yield of MEIS spectra was found to decrease with decreasing energy in contrast to conventional Rutherford backscattering spectrometry (RBS), due to the increase in neutralized particles. The HfxSiyO1−x−y spectra obtained by MEIS were corrected with a ratio of the simulated MEIS yield to measured yield for a virgin Si at each energy. The corrected HfxSiyO1−x−y spectra were analyzed and the compositions for 2 and 10 nm thick HfxSiyO1−x−y were obtained by MEIS. An interface layer was found to exist between the Si substrate and the HfxSiyO1−x−y layer.

Calcite crystals were prepared by dropping a saturated calcium carbonate aqueous solution on a substrate. The calcite crystals were grown with a growth rate of approximately 0.7 μm/day under the condition of aqueous temperature of 2 °C, aqueous concentration of 0.006 mol/l, and substrate temperature of 40 °C. When the calcite substrate was used, calcite crystals were grown epitaxially. Na 2 CO 3 and CaCl 2 aqueous solutions were dropped and reacted on the calcite crystal substrate with a CaCO 3 concentration of 0.06 mol/l, which is 10 times the saturated concentration at an aqueous temperature of 40 °C. Rhombohedral calcite was also deposited epitaxially with a growth rate of about 6.3 μm/day under this condition.

An in-situ reflectance monitor was used to improve the reproducibility and controllability of crystal growth by MBE (Molecular Beam Epitaxy). A DBR (Distributed Bragg Reflector) was grown using the in-situ monitor. The controllability of MBE has been remarkably improved and in-situ adjustment of the center wavelength of the stop band of DBR was also accomplished. The center wavelength, however, was found to shift 25 nm with decreasing substrate temperature after growth. This can be reasonably explained by the temperature change of the refractive indices. Therefore, it is necessary to set a target center wavelength at growth temperature, which is shifted form the designed center wavelength considering the temperature change of the refractive indices. (© 2006 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim)

We present the results of mapping observations with ISO of (O  )6 3µm, 145 µm, (N ) 122 µm, (C ) 158 µm, (Si  )3 5µm, and H2 9.66 µm line emissions for the Carina nebula, an active star-forming region in the Galactic plane. The observations were made for the central 40 � × 20 � area of the nebula, including the optically bright H  region and molecular cloud lying in front of the ionized gas. Around the center of the observed area is the interface between the H  region and the molecular cloud which creates a typical photodissociation region (PDR). The (C ) 158 µm emission shows a good correlation with the (O  )6 3µm emission and peaks around the H -molecular region interface. The correlated component has the ratio of (C ) 158 µ mt o (O )6 3µm of about 2.8. We estimate from the correlation that about 80% of (C ) emission comes from the PDR in the Carina nebula. The photoelectric heating efficiency estimated from the ratio of the ((C ) 158 µm + (O  )6 3µm) intensity to the total far-infrared intensity ranges from 0.06 to 1.2%. (O ) 145 µm is detected marginally at 10 positions. The average ratio of (O ) 145 µ mt o (O )6 3µm of these positions is about 0.09 ± 0.01 and is larger than model predictions. The observed (C ) 158 µ mt o (O )6 3µm ratio indicates a relatively low temperature (

We prepared Bi 2 Sr 2 Ca n −1 Cu n O y (Bi-based) superconducting whiskers by an Al 2 O 3 -seeded glassy quenched platelet (ASGQP) method. The whiskers grew from the Al 2 O 3 particles on the glassy quenched platelets in the ASGQP method. Scanning electron microscope results may indicate that the growth of the whiskers is caused by soaking out from the surface of the Al 2 O 3 particles. The optimum temperature for obtaining long whiskers was in the range of the temperatures from 860 to 875 °C. The width of the whiskers (in the direction of b -axis) was about 30–60 μm, and the surface was very flat. Using the method, we could prepare Bi-based whiskers with high growth rates, which was about 3 times as fast as that by previous method without any Al 2 O 3 seeds.

We prepared Bi 2 Sr 2 Ca n −1 Cu n O y (Bi-based) whiskers by an Al 2 O 3 -seeded glassy quenched platelet (ASGQP) method. As a result, the O 2 gas flow rates during the whisker growth did not affect the growth rates and the critical temperatures ( T c ) of the whiskers, whereas affected the critical current density ( J c ). The whisker with maximum J c was grown at the O 2 gas flow rate of 120 ml/min, and its value was about 2 × 10 5 A/cm 2 at 44 K, 0 T. The optimum O 2 gas flow rate for high J c Bi-based whiskers in the ASGQP method was about 120 ml/min.

The drain currents in PD-SOI-MOSFETs (partially depleted silicon-on-insulator metal oxide semiconductor field effects transistors) with and without body-tie structures have been measured using proton microprobe irradiation. The drain current characteristics in a PD-SOI-MOSFET with and without body-tie structures against ions irradiated at several points are also simulated by three dimensional device simulation. Both measurement and simulation show that the body-tie structure is effective for suppression of the abnormal current due to the floating body effects, though the body-tie structure cannot completely suppress the floating body effects. The measured drain currents for the PD-SOI-MOSFET without body-tie structures are by 4–20 times higher than those with body-tie structures, even though those with body-tie structures are higher than generated currents by proton microprobe irradiation. In the simulation the floating body effects in a PD-SOI-MOSFET with a body-tie structure are not observed in case of α-particles irradiation nearby the body contact electrode, while the floating body effects in a PD-SOI-MOSFET with a body-tie structure are observed in a case of another point irradiation.

Background Previously, in murine models of acid maltase deficiency (AMD), we demonstrated that intravenous administration of an improved adenovirus (Ad) vector encoding human acid alpha glucosidase (hGAA) resulted in liver transduction, followed by high-level hepatocyte-mediated secretion of hGAA into the plasma space. The hGAA secreted by the liver was taken up and targeted to muscle cell lysosomes. The levels of hGAA achieved by this approach resulted in clearance of lysosomal glycogen accumulations; in some muscle tissues the effect was prolonged (>6 months). We next wished to demonstrate whether this approach could be generalized across divergent species. To accomplish this goal, we determined whether a similar approach would also result in efficacy, but in a quail model of AMD. Methods An [E1-, E2b-]Ad vector encoding hGAA was intravenously injected into AMD quails. At several time points thereafter, plasma, liver, and multiple muscle tissues were assayed for evidence of hGAA gene expression, liver-mediated hGAA secretion, uptake of hGAA by skeletal muscles, and evidence of glycogen correction in AMD skeletal muscles. These results were compared with those obtained from mock-injected AMD or wild-type quails. Results Intravenous [E1-, E2b-]Ad/hGAA vector injection resulted in high-level liver transduction and hepatic secretion of precursor forms of hGAA. The hepatically secreted hGAA was found to not only be efficiently taken up by cardiac and skeletal muscles, but was also proteolytically cleaved and processed equivalently to the quail-GAA protein detected in wild-type quails. The observations suggest that the signals regulating muscle cell uptake (but not proteolytic cleavage) of lysosomal enzymes are conserved and recognized across divergent species of vertebrates. Importantly, once localized to skeletal muscle lysosomes, the hGAA was able to effectively clear the glycogen accumulations present in AMD quail muscles. Conclusions Adenovirus-mediated transduction of the hGAA gene, followed by hepatic secretion, uptake, and cross-correction of the pathologic glycogen accumulation noted in multiple muscles of both the AMD mouse and AMD quail, adds support to the notion that gene transfer strategies (Ad-mediated or other agents) targeting liver tissues with the hGAA gene are likely to be highly efficacious in humans affected by AMD. Copyright © 2002 John Wiley & Sons, Ltd.

We obtained maps of the central $40\arcmin \times 20\arcmin$ region of the Carina nebula, an active star forming region in the southern Milky Way, in various emission lines in the spectral range 43–197 μ m with the LWS on board the ISO. This paper reports on the results of [$\ion{O}{iii}$] 52, 88 μ m, [$\ion{N}{iii}$] 57 μ m, and [$\ion{N}{ii}$] 122 μ m lines. These ionized lines have been detected in the entire observed area, not only in the optically bright $\ion{H}{ii}$ region, but also in the molecular cloud direction and the region surrounding the Carina nebula. The electron density was derived from the [$\ion{O}{iii}$] lines and two distinct components were seen in the electron density map. One is a component with a density of 100–350 cm -3 , which encloses two $\ion{H}{ii}$ regions, Car I and Car II. The other is an extended diffuse component, in which the ratio of the [$\ion{O}{iii}$] lines is nearly at the low electron density limit ($n_{\rm e} -3 ). The observed [$\ion{N}{iii}$]/[$\ion{O}{iii}$] line intensity ratio also supports low electron density of the extended gas component. The diffuse component was detected in the entire observed region, which corresponds to ~30 pc at the distance of the Carina nebula. The present LWS observations of the ionic lines indicate the existence of highly-ionized diffuse gas of low electron density extending around the Carina nebula.

JEM-EUSO is a space science mission to explore the extreme energies and physics of the Universe. Its instrument will watch the darkside of the earth and will detect UV photons emitted from the extensive air shower caused by an ultra-high energy cosmic ray (UHECR above 1018 eV), or an extremely high energy cosmic ray (EHECR) particle (e.g. above about 1020 eV). Such a high-rigidity particle as the latter arrives almost in a straight line from its origin through the magnetic fields of our Milky Way Galaxy and is expected to allow us to trace the source location by its arrival direction. This can open the door to new astronomy with charged particles. In its 5 years of operation including the tilted mode, Extreme Universe Space Observatory an Japanese Experiment Module (JEM-EUSO) will detect at least 1000 events with E > 7 × 1019 eV with the Greisen–Zatsepin–Ku’zmin (GZK) suppression spectrum. It can determine the energy spectrum and source locations of GZK to super-GZK regions with a statistical accuracy of several percent. JEM-EUSO is planned to be deployed by H2 transfer vehicle (HTV) and will be attached to the Japanese Experiment Module/Exposure Facility (JEM/EF) of International Space Station. JAXA has selected JEM-EUSO as one of the mission candidates of the second phase utilization of JEM/EF for launch in the early-to-mid 2010 s.

Suppression of the floating body effects in partially depleted SOI–MOSFETs (silicon-on-insulator metal oxide semiconductor field effect transistors) with and without body contact electrodes has been investigated using nuclear microprobes with currents of 5–250 pA. Transient SOI–MOSFET behavior with and without body contact electrodes during ion irradiation by three-dimensional computer simulation has also been compared with that using nuclear microprobes. The floating body effects were observed in SOI–MOSFETs without body contact electrodes by nuclear microprobe irradiation, while those were suppressed in the SOI–MOSFETs with body contacts.

High-efficiency cadmium-free Cu(In,Ga)Se2 (CIGS) thin-film solar cells have been fabricated using a chemical bath deposition (CBD)-ZnS buffer layer, a wider band gap material than of conventional CBD-CdS. Energy dispersive X-ray microanalysis (EDX) revealed Zn interdiffusion in the CIGS thin film at the CBD-ZnS/CIGS solar cell interface, implying formation of a buried np junction at the surface of the CIGS film. The best cell to date yielded an active area efficiency of 17.2% after light soaking. This result suggests that CIGS solar cells with efficiencies as high as those fabricated using CdS buffer can be achieved even if Cd is not utilized.

Using the Mid-Infrared Spectrometer on board the Infrared Telescope in Space and the low-resolution grating spectrometer (PHT-S) on board the Infrared Space Observatory, we obtained 820 mid-infrared (5 to 12 $\mu$m) spectra of the diffuse interstellar medium (DIM) in the Galactic center, W51, and Carina Nebula regions. These spectra indicate that the emission is dominated by the unidentified infrared (UIR) emission bands at 6.2, 7.7, 8.6, and 11.2 $\mu$m. The relative band intensities (6.2/7.7 $\mu$m, 8.6/7.7 $\mu$m, and 11.2/7.7 $\mu$m) were derived from these spectra, and no systematic variation in these ratios was found in our observed regions, in spite of the fact that the incident radiation intensity differs by a factor of 1500. Comparing our results with the polycyclic aromatic hydrocarbons (PAHs) model for the UIR band carriers, PAHs in the DIM have no systematic variation in their size distribution, their degree of dehydrogenation is independent of the strength of UV radiation field, and they are mostly ionized. The latter finding is incompatible with past theoretical studies, in which a large fraction of neutral PAHs is predicted in this kind of environment. A plausible resolution of this discrepancy is that the recombination coefficients for electron and large PAH positive ion are by at least an order of magnitude less than those adopted in past theoretical studies. Because of the very low population of neutral state molecules, photoelectric emission from interstellar PAHs is probably not the dominant source of heating of the diffuse interstellar gas. The present results imply constant physical and chemical properties of the carriers of the UIR emission bands in the DIM., Comment: 13 pages, 6 figures. Accepted for publication in ApJ

The Carina Nebula region ( l ∼ 287° and b ∼ −0.6°) of about 20′ × 40′ has been observed by ISO/LWS with the spatial separation of 3′. Several emission lines, such as [OI] 63, 145 μ m, [OIII] 52, 88 μ m, [NIII] 57 μ m, [NII] 122 μ m and [CII] 157 μ m have been detected in the spectra of most observed positions. The LWS observations of the ionic lines indicate the presence of very low electron density gas ( n e −3 ) extended over the Galactic plane in a scale of kpc unambiguously for the first time.

The neurotoxic effects of 2,5-hexanedione (2,5-HD) were investigated using neurofilament (NF)-deficient (Quv) Japanese quail in comparison with normal Japanese quail. Both Quv and normal Japanese quail were inoculated intraperitoneally with 350 mg/kg/day 2,5-HD for 6 consecutive wk. The results of 2,5-HD exposure differed substantially between the 2 strains of Japanese quail. The 2,5-HD-exposed normal quail showed leg paralysis about 4 wk after initiation of dosing. Some treated normal quail fell into dysstasia and died of nutritional disturbances. Histologically, 2,5-HD-treated normal quail had NF-rich axonal swellings and degeneration in the distal parts of the peripheral nerves, spinal cord, and cerebellar peduncles. In contrast, 2,5-HD-injected Quv quail showed tonic convulsion, ataxia gait, severe quivering, and excitation about 2-3 days after administration. Some treated Quv birds died immediately after systemic tonic convulsion, probably because of asphyxia. Although all treated Quv quail showed neurologic signs, there were no recognizable 2,5-HD-induced lesions in the nervous system. After about 4-6 wk of dosing, 2,5-HD induced distal axonopathy in normal quail and acute neurotoxicity in Quv quail.

The effects of the diffusion control technique by inserting physical vapor deposition (PVD)-TiN film between poly-Si and CVD-TiN films on the properties of p-MISFETs using poly-Si/TiN/HfSiON gate stacks have been studied. This insertion was effective in suppressing the diffusion of Si from poly-Si to HfSiON and was able to reduce the Vth value by 0.12 V while keeping the equivalent oxide thickness and S value constant, when the thicknesses of the PVD and CVD-TiN films were 10 and 5 nm, respectively. Although too much ion implantation of fluorine into the substrate deteriorates S value and ION, it was verified that this diffusion control technique, in conjunction with a moderate substrate fluorine implantation, provided a reduction of Vth in pMIS without a deterioration of ION.

We have measured the specific heat on sputtered amorphous (a-) Ce x Ru 100− x alloys for 15⩽ x ⩽80 from 5 to 300 K. The C p /T nicely follows a function of γ + BT 2 above 8 K in each Ce concentration. In the Ce-rich side ( x ⩾67), the electronic specific coefficient γ shows a very large value ( γ =180 mJ/mol K 2 at x =80), suggesting formation of the heavy fermion (dense Kondo) state at low temperature. However, the γ decreases rapidly with decreasing the Ce concentration. In the Ru-rich side ( x γ becomes less than 10 mJ/mol K 2 and the superconductivity occurs. This γ -value is almost the same as for a-Ce 20− y La y Ru 80 in each La concentration, where the superconductivity occurs at the same superconducting temperature T c . We made the Kadowaki–Woods plot from the coefficient A of the T 2 term in resistivity and γ in the Ce-rich side. The value of A / γ 2 for present alloys shows about 3.5×10 −6 μ Ω cm(mol K 2 /mJ) 2 .

We report on specific CT and MR features in two cases of tumoral calcium pyrophosphate dihydrate deposition disease in the shoulder with unusually large tumors. CT revealed features that were specific to the disease. MR was useful for detecting the extent of the mass and for obtaining information on adjacent soft-tissue and bone-marrow changes

Acid alpha-glucosidase (GAA) deficiency causes Pompe disease, a lethal lysosomal glycogen storage disease for which no effective treatment currently exists. We investigated the endocytic process in deficient cells of human recombinant GAA produced in Chinese hamster ovary cells, and the potential of GAA-deficient Japanese acid maltase-deficient quail as a model for evaluating the enzyme replacement therapy for Pompe disease. After 24-h incubation with a single dose of recombinant enzyme, intracellular GAA and glycogen levels in deficient human fibroblasts were normalized, and this correction lasted for 7 d. The 110-kD precursor recombinant enzyme was processed to the 76-kD mature form within 24 h after uptake. Intracellular GAA levels in deficient quail fibroblasts and myoblasts were similarly corrected to their average normal levels within 24 h. Differences existed in the efficiency of endocytosis among subfractions of the enzyme, and among different cell types. Fractions with a larger proportion of precursor GAA were endocytosed more efficiently. Quail fibroblasts required a higher dose, 4200 nmol.h-1.mL-1 to normalize intracellular GAA levels than human fibroblasts, 1290 nmol.h-1.mL-1, whereas primary quail myoblasts required 2800 nmol.h-1.mL-1. In all three cell lines, the endocytosed enzyme localized to the lysosomes on immunofluorescence staining, and the endocytosis was inhibited by mannose 6-phosphate (Man-6-P) added to the culture medium. Despite structural differences in Man-6-P receptors between birds and mammals, these studies illustrate that Man-6-P receptor mediated endocytosis is present in quail muscle cells, and demonstrate the potential of acid maltase-deficient quail to test receptor mediated enzyme replacement therapy for Pompe disease.

Pompe disease is a fatal genetic muscle disorder caused by a deficiency of acid alpha-glucosidase (GAA), a glycogen degrading lysosomal enzyme. GAA-deficient (AMD) Japanese quails exhibit progressive myopathy and cannot lift their wings, fly, or right themselves from the supine position (flip test). Six 4-wk-old acid maltase-deficient quails, with the clinical symptoms listed, were intravenously injected with 14 or 4.2 mg/kg of precursor form of recombinant human GAA or buffer alone every 2-3 d for 18 d (seven injections). On day 18, both high dose-treated birds (14 mg/kg) scored positive flip tests and flapped their wings, and one bird flew up more than 100 cm. GAA activity increased in most of the tissues examined. In heart and liver, glycogen levels dropped to normal and histopathology was normal. In pectoralis muscle, morphology was essentially normal, except for increased glycogen granules. In sharp contrast, sham-treated quail muscle had markedly increased glycogen granules, multi-vesicular autophagosomes, and inter- and intrafascicular fatty infiltrations. Low dose-treated birds (4.2 mg/kg) improved less biochemically and histopathologically than high dose birds, indicating a dose-dependent response. Additional experiment with intermediate doses and extended treatment (four birds, 5.7-9 mg/kg for 45 d) halted the progression of the disease. Our data is the first to show that an exogenous protein can target to muscle and produce muscle improvement. These data also suggest enzyme replacement with recombinant human GAA is a promising therapy for human Pompe disease.