Restoration of holoceruloplasmin synthesis in LEC rat after infusion of recombinant adenovirus bearing WND cDNA

Wilson's disease, an autosomal recessive disorder, is characterized by the excessive accumulation of copper in the liver. WND (ATP7B) gene, which encodes a putative copper transporting P-type ATPase, is defective in the patients. To investigate the in vivo function of WND protein as well as its intracellular localization, WND cDNA was introduced to the Long-Evans Cinnamon rat, known as a rodent model for Wilson's disease, by recombinant adenovirus-mediated gene delivery. An immunofluorescent study and a subcellular fractionation study revealed the transgene expression in liver and its localization to the Golgi apparatus. Moreover, since the synthesis of holoceruloplasmin is disturbed in the Long-Evans Cinnamon rat, the plasma level of holoceruloplasmin, oxidase-active and copper-bound form, was examined to evaluate the function of WND protein with respect to the copper transport. Consequently, the appearance of holoceruloplasmin in plasma was confirmed by Western blot analysis and plasma measurements for the oxidase activity and the copper content. These findings indicate that introduced WND protein may function in the copper transport coupled with the synthesis of ceruloplasmin and that the Golgi apparatus is the likely site for WND protein to manifest its function.     

Left ventricular fibroma in an aged patient: report of a case

We report herein the case of a 77-year-old man with a left ventricular tumor originating from the papillary muscle of the left ventricular wall, in whom a successful tumor resection with mitral valve replacement was performed. The pathological diagnosis of the tumor was confirmed as cardiac fibroma. His postoperative course was uneventful and he is currently well with no signs of recurrence 2 years after surgery.     

Phase I study of orally administered UFT plus l-leucovorin

We describe the case of a 44-year-old woman with a delayed hemolytic transfusion reaction (DHTR). She had a history of two pregnancies and a blood transfusion, the details of which were unknown. At the time of her first vascular surgery on November 15, 1989, she received 1200 ml of crossmatch-compatible concentrated red blood cells (CRC). Before the first operation, screening for anti-RBC antibodies (Ab) was negative. At the time of the second admission on Feburary 15, 1996, anti-E Abs were detected by indirect antiglobulin test. She received 560 ml of E-antigen-negative, crossmatch-compatible, CRC for treatment of anemia on March 1 and 2, 1996. After this transfusion, total bilirubin (1.6 mg/dl) and lactate dehydrogenase (1355 IU/ml) were elevated on March 12, 1996. She had no evidence of clinical hemolysis. We suspected DHTR from these data, and therefore screened for anti-RBC Abs. Anti-E, Jka, Dia, Fyb, and S Abs were detected in blood samples obtained from the patient on March 12, 1996. Anti-E, Jka Dia, and S Abs were present more than 1 month and anti-Fyb Ab was disappeared at 18 days after transfusion.     

Emission under hypoxia: one-electron reduction and fluorescence characteristics of an indolequinone-coumarin conjugate

A characteristic feature of the reactivity of indolequinone derivatives, substituents of which can be removed by one-electron reduction under hypoxic conditions, was applied to the development of a new class of fluorescent probes for disease-relevant hypoxia. A reducing indolequinone parent molecule conjugated with fluorescent coumarin chromophores could suppress efficiently the fluorescence emission of the coumarin moieties by an intramolecular electron-transfer quenching mechanism and a conventional internal-filter effect. Under hypoxic conditions, however, the conjugate, denoted IQ-Cou, underwent a one-electron reduction triggered by X irradiation or the action of a reduction enzyme to release a fluorescent coumarin chromophore, whereupon an intense fluorescence emission with a maximum intensity at 420 nm was observed. The one-electron reduction of IQ-Cou was suppressed by molecular oxygen under aerobic conditions. IQ-Cou also showed intense fluorescence in a hypoxia-selective manner upon incubation with a cell lysate of the human fibrosarcoma cell line HT-1080. The IQ-Cou conjugate has several unique properties that are favorable for a fluorescent probe of hypoxia-specific imaging.     

Gas permeability of composite membranes

The permeability of a composite membrane consisting of a homogeneous layer and a porous layer has been derived theoretically by assuming that the permeation through the homogeneous layer obeys Fick's law and that permeation through the porous layer is free molecular flow. The activation energy of the flow is described by three-dimensionless parameters, ? = |P₁₂/|P₂, θ = d₁/d₂, and σ = |P₁/|P₂. |P₁₂, |P₁, and |P₂ are the permeability coefficients of the composite membrane, the homogeneous layer and the porous layer, respectively, d₁ and d₂ are the thickness of the two layers. Once these parameters are determined, information on the structure of the membrane can be obtained (i.e., the pore size and the pore density). The permeabilities of various gases through homogeneous polycarbonate membrane, neutron-irradiated, nonsodium hydroxide-etched polycarbonate membrane, and their composite membrane were tested. A two-layer series model, incorporating the effect of neutron irradiation which produces some nonpenetrating pores in the porous membrane layer, is proposed and agreed quite well with the experimental data.     

Characterization of novel biodegradable copolyesters prepared from glycolyzed products of poly(ethylene terephthalate)

Poly(ethylene terephthalate), PET reacted with various glycols in the presence of titanium tetraisopropoxide as a catalyst. The glycolyzed PET was fractionated into two parts—soluble and insoluble in CHCl₃—characterized and used as a raw material to synthesize copolyesters. The soluble part in the CHCl₃ part was a mixture of monomers and oligomers, and no melting points were observed. On the other hand, in the insoluble part a melting point was determined. The glycolyzed PET and copoly(succinic anhydride/ethylene oxide), PES were condensed to produce copolymers. The synthesized copolyesters were amorphous and hydrolyzed enzymatically due to a decrease in the crystallinity. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 84: 1838–1847, 2002; DOI 10.1002/app.10462     

Peptide Nucleic Acid Conjugated with Ruthenium‐Complex Stabilizing Double‐Duplex Invasion Complex Even under Physiological Conditions

Peptide nucleic acid (PNA) can form a stable duplex with DNA, and, accordingly, directly recognize double‐stranded DNA through the formation of a double‐duplex invasion complex, wherein a pair of complementary PNA strands form two PNA/DNA duplexes. Because invasion does not require prior denaturation of DNA, PNA holds great potential for in cellulo or in vivo applications. To broaden the applicability of PNA invasion, we developed a new conjugate of PNA with a ruthenium complex. This Ru–PNA conjugate exhibits higher DNA‐binding affinity, which results in enhanced invasion efficiency, even under physiological conditions.