The Ontogeny of Muscle Structure and Locomotory Function in the Long-Finned Squid Doryteuthis Pealeii

Understanding the extent to which changes in muscle form and function underlie ontogenetic changes in locomotory behaviors and performance is important in understanding the evolution of musculoskeletal systems and also the ecology of different life stages. We explored ontogenetic changes in the structure, myosin heavy chain (MHC) expression and contractile properties of the circular muscles that provide power for jet locomotion in the long-finned squid Doryteuthis pealeii. The circular muscle fibers of newly hatched paralarvae had different sizes, shapes, thick filament lengths, thin: thick filament ratio, myofilament organization and sarcoplasmic reticulum (SR) distribution than those of adults. Viewed in cross section, most circular muscle cells were roughly triangular or ovoid in shape with a core of mitochondria; however, numerous muscle cells with crescent or other unusual cross-sectional shapes and muscle cells with unequal distributions of mitochondria were present in the paralarvae. The frequency of these muscle cells relative to \u27normal\u27 circular muscle cells ranged from 1: 6 to 1: 10 among the 19 paralarvae we surveyed. The thick filaments of the two types of circular fibers, superficial mitochondria-rich (SMR) and central mitochondria-poor (CMP), differed slightly in length among paralarvae with thick filament lengths of 0.83+/-0.15 μm and 0.71+/-0.1μm for the SMR and CMP fibers, respectively (P 0.05; ANOVA). During ontogeny the thick filament lengths of both the CMP and SMR fibers increased significantly to 1.78+/-0.27 μm and 3.12+/-0.56 μm, respectively, in adults (P-1 (where L0 was the preparation length that generated the peak isometric stress), nearly twice that measured in other studies for the CMP fibers of adults. The mean peak isometric stress was 119+/-15mN mm-2 physiological cross section, nearly half that measured for the CMP fibers of adults. Reverse transcriptase-polymerase chain reaction analysis of paralarval and adult mantle samples revealed very similar expression patterns of the two known isoforms of squid MHC. The ontogenetic differences in the structure and physiology of the circular muscles may result in more rapid mantle movements during locomotion. This prediction is consistent with jet pulse durations observed in other studies, with shorter jet pulses providing hydrodynamic advantages for paralarvae

Histiocytoid cardiomyopathy and microphthalmia with linear skin defects syndrome: phenotypes linked by truncating variants in NDUFB11

Variants in NDUFB11, which encodes a structural component of complex I of the mitochondrial respiratory chain (MRC), were recently independently reported to cause histiocytoid cardiomyopathy (histiocytoid CM) and microphthalmia with linear skin defects syndrome (MLS syndrome). Here we report an additional case of histiocytoid CM, which carries a de novo nonsense variant in NDUFB11 (ENST00000276062.8: c.262C > T; p.[Arg88*]) identified using whole-exome sequencing (WES) of a family trio. An identical variant has been previously reported in association with MLS syndrome. The case we describe here lacked the diagnostic features of MLS syndrome, but a detailed clinical comparison of the two cases revealed significant phenotypic overlap. Heterozygous variants in HCCS (which encodes an important mitochondrially targeted protein) and COX7B, which, like NDUFB11, encodes a protein of the MRC, have also previously been identified in MLS syndrome including a case with features of both MLS syndrome and histiocytoid CM. However, a systematic review of WES data from previously published histiocytoid CM cases, alongside four additional cases presented here for the first time, did not identify any variants in these genes. We conclude that NDUFB11 variants play a role in the pathogenesis of both histiocytoid CM and MLS and that these disorders are allelic (genetically related)

A patient with persistent consolidation and a pulmonary mass

Community-acquired pneumonia is a common condition and 6–24% of patients will fail to improve as expected. We present a patient who was initially treated for community-acquired pneumonia but did not make the anticipated recovery. We explore potential differentials, and the investigation and management of the rare condition we subsequently diagnosed

Combination of pulsed laser ablation and inert gas condensation for the synthesis of nanostructured nanocrystalline, amorphous and composite materials

A new instrument combining pulsed laser ablation and inert gas condensation for the production of nanopowders is presented. It is shown that various nanostructured materials, such as regular metallic, semiconducting, insulating materials, complex high entropy alloys, amorphous alloys, composites and oxides can be synthesized. The unique variability of the experimental set-up is possible due to the reproducible control of laser power (pulse energy and repetition rate), laser ablation pattern on the target, and experimental conditions during the inert gas condensation, all of which can be controlled and optimized independently. Microstructure analysis of the as-prepared composite and amorphous Ni(60)Nb(40) nanopowders establishes the instrument's ability for the synthesis of materials with unique compositions and atomic structure. It is further shown that small variations of the synthesis parameters can influence materials properties of the final product, in terms of particle size, composition and properties. As an example, the laser power has been used to control the magnetic properties of amorphous Ni(60)Nb(40) nanopowders. A few selected examples of the manifold possibilities of the new synthesis apparatus are presented in this report together with detailed structural characterization of the produced nanopowders

Thermal conductivity of Barium Bismuthate at low temperatures

The perovskite BaBiO$_3$ crystallizes in a cubic structure and undergoes structural transitions toward lower symmetry phases upon cooling. The two low-temperature monoclinic phases are insulating, and the origin of this unexpected non-metallic character has been under debate. Both monoclinic phases exhibit tilting and breathing distortions, which are connected with the insulating nature of this compound and may have important effects on phononic heat conductivity. Here, we report the first thermal conductivity measurement, $\kappa$(T), in pristine polycrystalline BaBiO$_3$ from 1.5 K to 310 K. At low and intermediate temperatures, we observe features reminiscent of a glass-like behavior, whereas at high-temperatures we find a downturn - typical of a crystalline solid. We compare our findings with available data of other recently investigated perovskite oxides displaying similar temperature dependence

Syntheses, Crystal Structure, Electrocatalytic, and Magnetic Properties of the Monolanthanide-Containing Germanotungstates [Ln(H2_{2}O)n_{n}GeW11_{11}O39_{39}]5−^{5-} (Ln = Dy, Er, n = 4,3)

Two monolanthanide-containing polyanions based on monolacunary Keggin germanotungstates [Ln(H2O)nGeW11O39]5− (Ln = Dy, Er, n = 4,3) have been synthesized in simple one-pot synthetic procedure and compositionally characterized in solid state by single-crystal X-ray diffraction, powder X-ray diffraction, Fourier transform infrared spectroscopy, thermogravimetric analysis, and elemental analysis. Electronic absorption and emission spectra of the title compounds in solution were also studied. The [DyIII(H2O)4GeW11O39]5− Keggin POM exhibits a slow relaxation of magnetization. The cyclic voltammetry measurements and mass spectrometry were carried out to check the stability of the compounds in solution. Both polyanions prove efficient in the electrocatalytic reduction of nitrite. To our knowledge, this observation establishes the first example of electrocatalysis of nitrite reduction by all inorganic monolanthanidecontaining germanotungstates family

Predictors of outcome in a contemporary cardiac sarcoidosis population: Role of brain natriuretic peptide, left ventricular function and myocardial inflammation

Aims Cardiac sarcoidosis (CS) is a potentially fatal condition that varies in its clinical presentation. Here, we describe baseline characteristics at presentation along with prognosis and predictors of outcome in a sizable and deeply phenotyped contemporary cohort of CS patients. Methods and results Consecutive CS patients seen at one institution were retrospectively enrolled after undergoing laboratory testing, electrocardiogram, echocardiography, cardiac magnetic resonance (CMR) imaging and $^{18}$F‐flourodeoxyglucose positron emission tomography (FDG‐PET) at baseline. The composite endpoint consisted of all‐cause mortality, aborted sudden cardiac death, major ventricular arrhythmic events, heart failure hospitalization and heart transplantation. A total of 319 CS patients were studied (67% male, 55.4 ± 12 years). During a median follow‐up of 2.2 years (range: 1 month–11 years), 8% of patients died, while 33% reached the composite endpoint. The annualized mortality rate was 2.7% and the 5‐ and 10‐year mortality rates were 6.2% and 7.5%, respectively. Multivariate analysis showed serum brain natriuretic peptide (BNP) levels (hazard ratio [HR] 2.41, 95% confidence interval [CI] 1.34–4.31, p = 0.003), CMR left ventricular ejection fraction (LVEF) (HR 0.96, 95% CI 0.94–0.98, p < 0.0001) and maximum standardized uptake value of FDG‐PET (HR 1.11, 95% CI 1.04–1.19, p = 0.001) to be independent predictors of outcome. These findings remained robust for different patient subgroups. Conclusion Cardiac sarcoidosis is associated with significant morbidity and mortality, particularly in those with cardiac involvement as the first manifestation. Higher BNP levels, lower LVEF and more active myocardial inflammation were independent predictors of outcomes