Histopathological examination was performed on skeletal and diaphragmatic muscles from an 8-month-old male crossbred calf showing abnormal gait and tremor of the hindlimbs. There were numerous round ...fibres with centrally placed nuclei forming nuclear chains in longitudinal sections, associated with interstitial fibrosis or adipose tissue infiltration. On nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) staining, some muscle fibres in severe lesions showed a spoke-like appearance due to a radial arrangement of sarcoplasmic strands. Additionally, increased NADH-TR activity in the subsarcolemmal structures, appearingas ring-like or necklace-like forms, were observed. Transmission electron microscopy revealed dilated sarcoplasmic reticulum and variably shaped electron-dense inclusions consisting of myofibrillar streams. Another prominent feature was the existence of numerous nemaline rods within muscle fibres; these were stained red by Gomori's trichrome stain. Immunohistochemistry revealed that the nemaline rods showed strong immunoreactivity with α-actinin and desmin antibodies. Electron microscopically, these structures were composed of dense-homogeneous material and continuous with the Z disk. The case was diagnosed as centronuclear myopathy with increased nemaline rods.
Summary
Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease that occurs in association with lichen planus (LP). This report describes a 59‐year‐old Japanese female patient with ...LPP. The patient first showed LP lesions on her hands, and subsequently developed bullae on her extremities and erosions of the oral mucosa. The patient's serum was positive for IgG autoantibodies against the BP180 NC16a domain, the BP180 C‐terminal domain and desmoglein‐1. However, a serum sampled one and a half years before the diagnosis of LPP was negative for autoantibodies against BP180 NC16a and BP180 C‐terminal domains. These findings strongly suggest that the damage to the basal cells in the LP lesions exposed a sequestered antigen or formed neoantigens, leading to the production of pathogenic autoantibodies for LPP. Most of the previous cases of LPP have produced autoantibodies to the NC16a domain of BP180. This is the first case in which autoantibodies to the C‐terminal domain of BP180 were detected. The oral mucosal symptoms in this case may have been caused by autoantibodies to the BP180 C‐terminal domain.
What's already known about this topic?
Damage to the basal cells in lichen planus may expose sequestered antigens or produce neoantigens, leading to autoantibody production.
Autoantibodies to the NC16a domain of BP180 have been detected in most cases of lichen planus pemphigoides.
What does this study add?
This report demonstrates the presence of circulating autoantibodies to BP180 only after the appearance of bullae, suggesting the role of sequestered antigen exposure or neoantigens in the pathogenesis of the disease.
The present case showed autoantibodies to the C‐terminal domain of BP180, which has never been reported, and showed that they may cause oral mucosal lesions.
Burning velocity has been measured using the spherical-vessel (SV) method for four hydrofluorocarbons (HFCs), i.e., difluoromethane (HFC-32), 1,1,2-trifluoroethane (HFC-143), 1,1,1-trifluoroethane ...(HFC-143a), and 1,1-difluoroethane (HFC-152a). Experiments were conducted for initial pressures in the range 78–108 kPa and initial temperatures in the range 280–330 K, over wide ranges of HFC–air equivalence ratios. The burning velocities were determined from the rate of pressure increase by applying a spherical-flame propagation model. Flame propagation behavior was observed in a cylindrical vessel equipped with optical windows by employing schlieren photography. It was found that the values of burning velocity derived from the spherical-vessel method are in good agreement with the ones obtained with schlieren method. It is found that the SV method is adequate for determining the burning velocity for weakly flammable HFCs as well as for moderately flammable compounds. The burning velocity of each HFC was obtained as a function of temperature, pressure, and equivalence ratio. The maximum burning velocities of HFC-32, HFC-143, HFC-143a, and HFC-152a were determined to be 6.7, 13.1, 7.1, and 23.6 cm s
−1, respectively. The maximum burning velocity occurred for slightly fuel-rich concentrations. The magnitude of the burning velocity is strongly dependent on the ratio of H atoms to F atoms in the HFC molecules.
Background
Mutations in FLG, which encodes profilaggrin, cause ichthyosis vulgaris (IV) and are an important predisposing factor for atopic dermatitis (AD). IV shows autosomal hemidominant ...(semidominant) inheritance, and patients with bi‐allelic FLG mutations tend to have severe IV phenotypes. However, the effect of bi‐allelic FLG mutations on AD incidence and severity remains a subject of controversy.
Objective
In this study, we studied individuals with bi‐allelic null FLG mutations to elucidate the effect of bi‐allelic FLG mutations on AD incidence and severity.
Methods
Six individuals with bi‐allelic FLG null mutations from three families of IV/AD were investigated. We report the detailed clinical features of the individuals. The phenotype was confirmed by the clinical examinations and the severity of IV and AD was scored using ichthyosis score and Eczema Area and Severity Index (EASI).
Result
It was found that five of the six patients had severe IV, and the remaining patient showed moderate IV. Two of the six had moderate AD and three of the six had mild AD. The remaining patient had no AD.
Conclusion
Our results suggest that individuals with bi‐allelic FLG mutations do not always have severe AD and confirm that not all individuals with bi‐allelic FLG mutations have AD.
The kinetics and mechanism of oxidation of CF3CHFOCH3 was studied using an 11.5-dm3 environmental reaction chamber. OH radicals were produced by UV photolysis of an O3−H2O−He mixture at an initial ...pressure of 200 Torr in the chamber. The rate constant of the reaction of CF3CHFOCH3 with OH radicals (k 1) was determined to be (1.77 ± 0.69) × 10-12 exp(−720 ± 110)/T cm3 molecule-1 s-1 by means of a relative rate method at 253−328 K. The mechanism of the reaction was investigated by FT-IR spectroscopy at 298 K. CF3CHFOC(O)H, FC(O)OCH3, and COF2 were determined to be the major products. The branching ratio (k 1a/k 1b) for the reactions CF3CHFOCH3 + OH → CF3CHFOCH2 • + H2O (k 1a) and CF3CHFOCH3 + OH → CF3CF•OCH3 + H2O (k 1b) was estimated to be 4.2:1 at 298 K from the yields of CF3CHFOC(O)H, FC(O)OCH3, and COF2. The rate constants of the reactions of CF3CHFOC(O)H (k 2) and FC(O)OCH3 (k 3) with OH radicals were determined to be (9.14 ± 2.78) × 10-13 exp(−1190 ± 90)/T and (2.10 ± 0.65) × 10-13 exp(−630 ± 90)/T cm3 molecule-1 s-1, respectively, by means of a relative rate method at 253−328 K. The rate constants at 298 K were as follows: k 1 = (1.56 ± 0.06) × 10-13, k 2 = (1.67 ± 0.05) × 10-14, and k 3 = (2.53 ± 0.07) × 10-14 cm3 molecule-1 s-1. The tropospheric lifetimes of CF3CHFOCH3, CF3CHFOC(O)H, and FC(O)OCH3 with respect to reaction with OH radicals were estimated to be 0.29, 3.2, and 1.8 years, respectively.
We evaluated timing jitter based on a time-to-digital converter (TDC) having sub-pico seconds time resolution. The measured jitter at liquid helium temperature was 65 fs for an optimally damped ...simple junction composing a conventional Josephson transmission line fabricated with the NEC standard process. The temperature dependence and shunt resistance dependence of jitter indicate that the jitter originates from thermal noise generated at the shunt resistor of the junction. We also found that the timing jitter of a balanced comparator is much larger than that of a JTL. The jitter of a balanced comparator is independent of temperature. This means that the jitter of a balanced comparator does not arise from thermal noise.
The atmospheric chemistry of (CF3)2CHOCH3, a possible HCFC/HFC alternative, was studied using a smog chamber/FT-IR technique. OH radicals were prepared by the photolysis of ozone in a 200-Torr ...H2O/O3/O2 gas mixture held in an 11.5-dm3 temperature-controlled chamber. The rate constant, k 1, for the reaction of (CF3)2CHOCH3 with OH radicals was determined to be (1.40 ± 0.28) × 10-12 exp(−550 ± 60)/T cm3 molecule-1 s-1 by means of a relative rate method at 253−328 K. The value of k 1 at 298 K was (2.25 ± 0.04) × 10-13 cm3 molecule-1 s-1. The random errors are reported with ±2 standard deviations, and potential systematic errors of 15% could increase k 1. In considering OH-radical reactions, we estimated the tropospheric lifetime of (CF3)2CHOCH3 to be 2.0 months using the rate constant at 288 K. The degradation mechanism of (CF3)2CHOCH3 initiated by OH radicals was also investigated using FT-IR spectroscopy at 298 K. Products (CF3)2CHOC(O)H, CF3C(OH)2CF3, CF3C(O)OCH3, and COF2 were identified and quantified. The branching ratio, k 1a/k 1b, was estimated to be 2.1:1 for reactions (CF3)2CHOCH3 + OH → (CF3)2CHOCH2 •+ H2O (k 1a) and (CF3)2CHOCH3 + OH → (CF3)2C•OCH3 + H2O (k 1b).
Burning velocity measurements of nitrogen-containing compounds, i.e., ammonia (NH
3), methylamine (CH
3NH
2), ethylamine (C
2H
5NH
2), and propylamine (C
3H
7NH
2), were carried out to assess the ...flammability of potential natural refrigerants. The spherical-vessel (SV) method was used to measure the burning velocity over a wide range of sample and air concentrations. In addition, flame propagation was directly observed by the schlieren photography method, which showed that the spherical flame model was applicable to flames with a burning velocity higher than approximately 5
cm
s
−1. For CH
3NH
2, the nozzle burner method was also used to confirm the validity of the results obtained by closed vessel methods. We obtained maximum burning velocities (
S
u0,max) of 7.2, 24.7, 26.9, and 28.3
cm
s
−1 for NH
3, CH
3NH
2, C
2H
5NH
2, and C
3H
7NH
2, respectively. It was noted that the burning velocities of NH
3 and CH
3NH
2 were as high as those of the typical hydrofluorocarbon refrigerants difluoromethane (HFC-32,
S
u0,max
=
6.7
cm
s
−1) and 1,1-difluoroethane (HFC-152a,
S
u0,max
=
23.6
cm
s
−1), respectively. The burning velocities were compared with those of the parent alkanes, and it was found that introducing an NH
2 group into hydrocarbon molecules decreases their burning velocity.
