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3'-Azido-3'-deoxythymidine (AZT), an antiretroviral drug, is often adopted in the therapy for human immunodeficiency virus (HIV) infection, and the characteristics of AZT transport at the blood-testis barrier (BTB) were investigated in this study. In the integration plot analysis that evaluates the transport activity in vivo, the apparent influx clearance of [

Deprotonatable ionic-liquid surfactant cation of 1-dodecylimidazolium (C12im) was successfully hybridized with polyoxovanadate anion to obtain conductive inorganic-organic hybrid crystal. Single crystal X-ray analysis revealed that the hybrid crystal contained the alternate stacking of decavanadate (V10) layers and interdigitated C12im layers. Four C12im cations were associated with one diprotonated V10 anion, and the two diprotonated V10 anions formed a dimer species. Anhydrous proton conductivity derived from the dissociative protons of the hybrid crystal was estimated to be 4.6 × 10−5 S cm−1 at 373 K by alternating current (AC) impedance spectroscopy.

Proton-conducting materials working under anhydrous or less-humidified atmosphere are significant for improving the energy efficiency of fuel-cell batteries. Here, proton-containing inorganic-organic hybrid crystals were successfully constructed as single crystals with polyoxovanadate cluster and structure-directing surfactants. Decavanadate ([V10O28]6‒, V10) anion having proton affinity was employed to hybridize with alkyltrimethylammonium ([CnH2n+1N(CH3)3]+, Cn (n = 12, 14, 16)) cations. All the hybrid crystals were homologues composed from alternate stacking of V10 monolayers and Cn interdigitated bilayers. Inside the V10 monolayers, diprotonated V10 anions were arranged to form one-dimensional infinite chains connected through O‒H•••O hydrogen bonds. These hybrid crystals exhibited anhydrous proton conductivity at intermediate temperatures (373‒433 K (100‒160 °C)). A moderate anhydrous conductivity of 2.9 × 10–4 S cm–1 was observed for C16-V10 hybrid crystals at 373 K (100 °C).

Monovalent metal cations (Ag+, K+, and Cs+) were successfully introduced into inorganic-organic hybrid crystals composed from β-type octamolybdate (β-Mo8) and hexadecylpyridinium (C16py). The introduction of metal cations was induced by the molecular isomerization from α-Mo8 to β-Mo8 anion. All hybrid crystals consisted of alternate stacking of β-Mo8 inorganic and C16py organic layers. In the inorganic layers, the metal cations and β-Mo8 anions were connected to form one- or two-dimensional arrangement, which depended on the ionic radius of metal cations.

Polyoxomolybdate-surfactant hybrid layered crystals were synthesized by using single-tailed alkyltrimethylammonium ([C n H 2 n +1 N(CH 3 ) 3 ] + (C n ), n = 8, 10, 12, 14, 16, and 18) cations. The crystal structures consisted of alternate stacking of octamolybdate ([Mo 8 O 26 ] 4− , Mo 8 ) anionic layers and surfactant cationic layers. The layered distance of the hybrid crystals became longer from 18.5 A to 26.5 A with an increase in alkyl chain length. Interestingly, the molecular structures of Mo 8 , which has several isomers, depended on the alkyl chain length of the employed surfactants. Shorter surfactant (C 8 and C 10 ) formed hybrid crystals containing β-type Mo 8 isomer, while surfactant with longer alkyl chain (C 12 , C 14 , C 16 , and C 18 ) gave crystals containing δ-type Mo 8 isomer. This structural controllability will lead to the precise functional control in the polyoxomolybdate-surfactant hybrid layered crystals.

Synthetic bilayers of double-chained surfactants were successfully constructed by using polyoxomolybdates as inorganic–organic hybrid crystals. Dialkyldimethylammonium ([(CnH2n+1)2N(CH3)2]+, (Cn)2; n = 10, 12, 14, 16) cations were employed to hybridize with octamoybdate ([Mo8O26]4–, Mo8) anion. The obtained crystals were homologues which possessed distinct layered structures composed from alternate stacking of Mo8 monolayers and (Cn)2 bilayers. The layered distances were controllable with an increase in the alkyl chain length of the (Cn)2 cation. Each alkyl chain of (Cn)2 was interdigitated to result in rather unique conformations for the (Cn)2 cations packed in the solid states. Inside the inorganic monolayers, the Mo8 anions were associated with sodium ions to form two-dimensional network, which could be promising for sodium-ion conductive materials.

Information on the phenotypic variations seen in patients with type 3 (chronic neuronopathic) Gaucher disease (GD) is still limited compared with type 1 GD. We retrospectively investigated the clinical features of 42 Japanese patients with type 3 GD. The 42 patients classified as type 3 fell into two groups: those diagnosed as having type 3 GD at diagnosis (group A; n = 24) and those thought to have type 1 at diagnosis but who later developed neurological symptoms (group B; n = 18). The genotype of group A patients varied widely; however, L444P/L444P and L444P/F213I genotypes accounted for 83% in group B. All the patients who did not receive enzyme replacement with alglucerase or imiglucerase (4 in group A, 2 in group B) died. Nineteen patients received enzyme replacement in group A; however, 7 of these died despite the therapy. On the other hand, 14 patients received enzyme replacement alone in group B and 13 of them survived. Among the ERT-treated patients who survived, only one of 12 in group A and 12 out of 13 in group B can walk unaided. In conclusion, some Japanese GD patients who are thought to have type 1 at diagnosis develop neurological symptoms during their clinical course, and careful observation is essential for patients with characteristic genotypes. Moreover, enzyme replacement alone might not have a sufficient effect on the early onset neurological symptoms in type 3 patients. A different treatment strategy is needed to improve the prognosis of these patients.

The microstructures of aggregates of heteropoly compounds Cs3PW12O40 and (NH4)3PW12O40 precipitated at various temperatures were characterized by SEM, XRD, TEM, ED, and N2 adsorption. The microstructures were remarkably different according to the precipitation temperature and the kind of cation. Cs3PW12O40 precipitated at 298 K formed dispersed or loosely aggregated fine particles of ca. 10 nm with random crystal orientation. As the temperature increased to 368 K, Cs3PW12O40 self-assembled to form microporous aggregates in which the crystal planes of microcrystallites were oriented unidirectionally, but were not much connected epitaxially with each other. (NH4),PW12O40 precipitated at 273 K formed round aggregates similar to Cs3PW12O40 precipitated at 368 K, while the NH4 salt precipitated at 368 K formed dodecahedral aggregates in which microcrystallites were epitaxially connected with each other (epitaxial self-assembly). These observations indicate that microstructure is controlled by the solubility at the precipitating conditions; with increasing precipitation temperature or decreasing ionic radii of cation, the microstructure of aggregates varies from the random aggregates to unidirectionally oriented microcrystallites and the epitaxial connection between microcrystallites develops.

ABSTRACT: Gaucher disease is caused by a deficiency of glucocerebrosidase, resulting in hepatosplenomegaly, pancytopenia, growth retardation and skeletal involvement. We analyzed data on genotype and key clinical parameters in 35 Japanese patients with Gaucher disease type 1. Our data demonstrated that over 60% of patients had onset of Gaucher disease signs/symptoms at less than 5 years. Sixty percent and 46% of evaluable patients were splenectomized and developed severe bone involvement, respectively. Within mean follow-up periods of 8 years and 4 months, mean relative height and weight, severity score index and platelet count all worsened to a highly significant degree. These data suggest that type 1 Gaucher disease tends to be severe and progressive in Japanese patients, most of whom would be suitable for treatment and might indeed require earlier and more aggressive therapy.

Fabrication condition and photoluminescence behavior of decatungstoeuropate/dimethyldioctadecylammonium (EuW10/DODA) Langmuir–Blodgett (LB) monolayers were investigated. Stable Langmuir monolayer were formed on the EuW10 aqueous solution within the concentration range of 0.84–3.1 μmol dm −3 . The monolayer formed on the solution was successfully deposited onto quartz substrates with nearly ideal transfer ratio. The presence of EuW10 anion was confirmed by UV–vis spectroscopy. Strong red emission derived from Eu 3+ was clearly observed even for the EuW10/DODA LB monolayers when excited by 266 nm laser light. The intensity of 5 D 0 → 7 F 4 emission was unusually high, suggesting the effect of two-dimensional organization of EuW10 anions in the monolayer.