2,4,8,10-Tetra­oxa-3,9-dithia­spiro­[5.5]undecane 3,9-dioxide

The asymmetric unit of the title compound, C5H8O6S2, consists of two spiro­[5.5]undecane mol­ecules. The nonplanar six-membered rings adopt chair conformations. In the crystal structure, weak inter­molecular C—H⋯O inter­actions, together with close O⋯S contacts in the range 3.308 (3)–3.315 (3) Å, stabilize the packing

(4,4′-Di-tert-butyl-2,2′-bipyridine-κ2 N,N′)bis­(nitrato-κ2 O,O′)copper(II)

In the crystal of the title compound, [Cu(NO3)2(C18H24N2)], the CuII ion is coordinated by two N atoms of the bipyridine ligand and four O atoms from the two nitrate anions in a distorted octahedral fashion. The dihedral angle between the planes of the two pyridine rings is 11.52 (10)°. In the crystal structure, weak C—H⋯O inter­actions may help to establish the packing

2 Novel deletions of the sterol 27-hydroxylase gene in a Chinese Family with Cerebrotendinous Xanthomatosis

<p>Abstract</p> <p>Background</p> <p>Cerebrotendinous xanthomatosis (CTX) is a rare lipid-storage disease. We investigated the clinic manifestation, histopathology and sterol 27-hydroxylase gene (CYP27A1) in a Chinese family with Cerebrotendinous Xanthomatosis (CTX).</p> <p>Case Presentation</p> <p>A 36-year-old female with typical CTX clinical manifestation had Spindle-shaped lipid crystal clefts in xanthomas and "onion-like demyelination" in sural nerve. The patient was compound heterozygote carrying two deletions in exon 1 (c.73delG) and exon 2 (c.369_375delGTACCCA). The family memebers were carriers.</p> <p>Conclusions</p> <p>A Chinese family with Cerebrotendinous Xanthomatosis had typical clinical manifestation. CYP27A1 mutations were found in the proband and all other family members.</p

Lysimachia danxiashanensis, a new species of Primulaceae from Guangdong, China

Lysimachia danxiashanensis, a new Primulaceae species, endemic to the Danxia landscape in Guangdong Province, China, is described and illustrated. This new species is morphologically similar to L. pseudohenryi, L. phyllocephala, L. congestiflora and L. kwangtungensis, but it differs from the similar species by its purplish-red plants, petiole without wings, calyx with orange glandular and the corolla margin serrated on upper half with orange-red glandular punctates. This new species belongs to Lysimachia subgen. Lysimachia sect. Nummularia. Phylogenetic analysis confirmed that L. danxiashanensis is a distinct clade, based on the combined data of ITS and rbcL sequences. The conservation status of the new species was evaluated as Endangered (EN) according to IUCN Red List Categories and Criteria

Ca2+ Cycling in Heart Cells from Ground Squirrels: Adaptive Strategies for Intracellular Ca2+ Homeostasis

Heart tissues from hibernating mammals, such as ground squirrels, are able to endure hypothermia, hypoxia and other extreme insulting factors that are fatal for human and nonhibernating mammals. This study was designed to understand adaptive mechanisms involved in intracellular Ca2+ homeostasis in cardiomyocytes from the mammalian hibernator, ground squirrel, compared to rat. Electrophysiological and confocal imaging experiments showed that the voltage-dependence of L-type Ca2+ current (ICa) was shifted to higher potentials in ventricular myocytes from ground squirrels vs. rats. The elevated threshold of ICa did not compromise the Ca2+-induced Ca2+ release, because a higher depolarization rate and a longer duration of action potential compensated the voltage shift of ICa. Both the caffeine-sensitive and caffeine-resistant components of cytosolic Ca2+ removal were more rapid in ground squirrels. Ca2+ sparks in ground squirrels exhibited larger amplitude/size and much lower frequency than in rats. Due to the high ICa threshold, low SR Ca2+ leak and rapid cytosolic Ca2+ clearance, heart cells from ground squirrels exhibited better capability in maintaining intracellular Ca2+ homeostasis than those from rats and other nonhibernating mammals. These findings not only reveal adaptive mechanisms of hibernation, but also provide novel strategies against Ca2+ overload-related heart diseases

Genomic Dissection of Bipolar Disorder and Schizophrenia, Including 28 Subphenotypes

publisher: Elsevier articletitle: Genomic Dissection of Bipolar Disorder and Schizophrenia, Including 28 Subphenotypes journaltitle: Cell articlelink: https://doi.org/10.1016/j.cell.2018.05.046 content_type: article copyright: © 2018 Elsevier Inc

Research progress of pathophysiological concepts in cerebral small vessel disease

The current definition of cerebral small vessels refers to all vascular structures ranging from 5 μ m to 2 mm in diameter in the brain parenchyma and they encompass small arteries, arterioles, capillaries, venules and small veins. Different diameters of cerebral small vessels have different distribution, and predilection to different types of pathophysiological process. Cerebral small vessel disease (cSVD), including lacunar infarct (LACI), white matter hyperintensity (WMH) and cerebral microbleeds (CMBs), have different pathophysiological mechanisms and risk factors. Arterial stiffness is independently associated with all components of cSVD. DOI: 10.3969/j.issn.1672-6731.2016.11.007</p

Coupled Thermo-Mechanical Phase-Field Modeling to Simulate the Crack Evolution of Defective Ceramic Materials under Flame Thermal Shock

Crack propagation in ceramics is a highly quick, complex, and nonlinear process that occurs under thermal shock. It is challenging to directly observe the evolution process of cracks in experiments due to the high speed and unpredictability of crack propagation. Based on the phase-field fracture method, a phase-field numerical model combined with thermal and mechanical damage is established to analyze the crack propagation path, velocity, and morphology of pre-cracked ceramic plates under flame thermal shock loading. This research primarily focuses on the impact of prefabricated crack angle and length on crack propagation. According to the findings of the numerical simulation, ceramic plates with varied prefabricated crack angles are loaded via flame thermal shock, and thermal stress is caused by the rapid rise in the temperature difference between the top edge and the inside of the ceramic plate. Hence, the crack propagation rate seems to be quick at first, and then, slows down when the wing-like cracks at the crack tips spread to both ends. The crack tip on the side closer to the flame thermal loading is more likely to generate wing-shaped cracks as the length of the pre-existing crack increases. However, the crack tip on the side further away from the flame thermal loading exhibits the reverse tendency. The complex evolution process of crack initiation, propagation, and coalescence in ceramic materials brought on by flame thermal shock can be predicted by the thermo-mechanical coupled phase-field model, which is a valuable reference for designing and optimizing the thermal shock resistance and mechanical failure prediction of ceramic materials