BACKGROUND: The skin changes of X-linked recessive ichthyosis are cnused by the deficiency of the enzyme steroid sulfatase, which usually results from deletions of this gene in Caucasian populations. OBJECTIVE AND MEHTODS: To disgnose X-linked recessive ichthyosis and detect its carrier, we have investigated distinctive gene deletion and measured gene dosage of steroid sulfatase gene by southern blot hybridization in Korean patients with X-linked recessive ichthyosis. RESULTS: Patients from 8 of 9 unrelated families exhibited deletions, if the steroid sulfatase gene. Of 6 families showing a family history compatible with X-linked recessive inheritance, One family exhibited a normal pattern of hybridization. All but one family showed deletion of steroid sulfatase gene. All three patients lacking a fami1y history of the disease exhibited gene deletions. The ratio of the steroid sulfatsse specific band density to the Factor VIII specific band density was measured in 8 obligate carriers using a laser densitometer. The average ratio exhibited by the car riers was less than half that of normal women. Conclusian: These results suggest that the X-linked recessive ichth osis patient and its carrier can also be diagnosed and detected by Southern blot hybridization of steroid sulfatase gene in Korea.
Blotting, Southern* ; Diagnosis* ; Factor VIII ; Female ; Gene Deletion ; Gene Dosage ; Humans ; Ichthyosis* ; Korea ; Skin ; Steryl-Sulfatase ; Wills

99mTc tricarbonyl glycine monomers, trimers, and pentamers were synthesized and evaluated for their radiolabeling and in vivo distribution characteristics. We synthesized a 99mTc-tricarbonyl precursor with a low oxidation state (I). 99mTc(CO)3(H2O)3 + was then made to react with monomeric and oligomeric glycine for the development of bifunctional chelating sequences for biomolecules. Labeling yields of 99mTc-tricarbonyl glycine monomers and oligomers were checked by high-performance liquid chromatography. The labeling yields of 99mTc-tricarbonyl glycine and glycine oligomers were more than 95%. We evaluated the characteristics of 99mTc-tricarbonyl glycine oligomers by carrying out a lipophilicity test and an imaging study. The octanol-water partition coefficient of 99mTc tricarbonyl glycine oligomers indicated hydrophilic properties. Single-photon emission computed tomography imaging of 99mTc-tricarbonyl glycine oligomers showed rapid renal excretion through the kidneys with a low uptake in the liver, especially of 99mTc tricarbonyl triglycine. Furthermore, we verified that the addition of triglycine to prototype biomolecules (AGRGDS and RRPYIL) results in the improvement of radiolabeling yield. From these results, we conclude that triglycine has good characteristics for use as a bifunctional chelating sequence for a 99mTc-tricarbonyl-based biomolecular imaging probe.
Chromatography, Liquid ; Glycine ; Kidney ; Lifting ; Liver ; Oligopeptides ; Pentamidine ; Tomography, Emission-Computed

The process of drug discovery and development requires substantial resources and time. The drug industry has tried to reduce costs by conducting appropriate animal studies together with molecular biological and genetic analyses. Basic science research has been limited to in vitro studies of cellular processes and ex vivo tissue examination using suitable animal models of disease. However, in the past two decades new technologies have been developed that permit the imaging of live animals using radiotracer emission, X-rays, magnetic resonance signals, fluorescence, and bioluminescence. The main objective of this review is to provide an overview of small animal molecular imaging, with a focus on nuclear imaging (single photon emission computed tomography and positron emission tomography). These technologies permit visualization of toxicodynamics as well as toxicity to specific organs by directly monitoring drug accumulation and assessing physiological and/or molecular alterations. Nuclear imaging technology has great potential for improving the efficiency of the drug development process.
Animals ; Drug Discovery ; Drug Industry ; Electrons ; Fluorescence ; Magnetic Resonance Spectroscopy ; Models, Animal ; Molecular Imaging ; Tomography, Emission-Computed

The selective targeting of an integrin alphavbeta3 receptor using radioligands may enable the assessment of angiogenesis and integrin alphavbeta3 receptor status in tumors. The aim of this research was to label a peptidomimetic integrin alphavbeta3 antagonist (PIA) with 99mTc(CO)3 and to test its receptor targeting properties in nude mice bearing receptor-positive tumors. PIA was reacted with tris-succinimidyl aminotriacetate (TSAT) (20 mM) as a PIA per TSAT. The product, PIA-aminodiacetic acid (ADA), was radiolabeled with [99mTc(CO)3(H2O)3](+1), and purified sequentially on a Sep-Pak C-18 cartridge followed by a Sep-Pak QMA anion exchange cartridge. Using gradient C-18 reverse-phase HPLC, the radiochemical purity of 99mTc(CO)3-ADA-PIA (retention time, 10.5 min) was confirmed to be > 95%. Biodistribution analysis was performed in nude mice (n = 5 per time point) bearing receptor-positive M21 human melanoma xenografts. The mice were administered 99mTc(CO)3-ADA-PIA intravenously. The animals were euthanized at 0.33, 1, and 2 hr after injection for the biodistribution study. A separate group of mice were also co-injected with 200 microg of PIA and euthanized at 1 hr to quantify tumor uptake. 99mTc(CO)3-ADA-PIA was stable in phosphate buffer for 21 hr, but at 3 and 6 hr, 7.9 and 11.5% of the radioactivity was lost as histidine, respectively. In tumor bearing mice, 99mTc(CO)3-ADA-PIA accumulated rapidly in a receptor-positive tumor with a peak uptake at 20 min, and rapid clearance from blood occurring primarily through the hepatobiliary system. At 20 min, the tumor-to-blood ratio was 1.8. At 1 hr, the tumor uptake was 0.47% injected dose (ID)/g, but decreased to 0.12% ID/g when co-injected with an excess amount of PIA, indicating that accumulation was receptor mediated. These results demonstrate successful 99mTc labeling of a peptidomimetic integrin antagonist that accumulated in a tumor via receptor-specific binding. However, tumor uptake was very low because of low blood concentrations that likely resulted from rapid uptake of the agent into the hepatobiliary system. This study suggests that for 99mTc(CO)3-ADA-PIA to be useful as a tumor detection agent, it will be necessary to improve receptor binding affinity and increase the hydrophilicity of the product to minimize rapid hepatobiliary uptake.
Animals ; Chromatography, High Pressure Liquid ; Histidine ; Humans ; Hydrophobic and Hydrophilic Interactions ; Integrin alphaVbeta3 ; Melanoma ; Mice ; Mice, Nude ; Radioactivity ; Succinimides ; Transplantation, Heterologous ; Ursidae

No abstract available.
Hepacivirus ; Hepatitis C* ; Hepatitis*

PURPOSE: To assess the agreement and compare the performance of glaucoma diagnosis of peripapillary retinal nerve fiber layer (RNFL) thickness measurements between two different spectral-domain optical coherence tomography (SD-OCT) devices. METHODS: Eighty nine eyes of 56 patients with glaucoma and 42 eyes of 25 healthy individuals were imaged with Cirrus and Spectralis OCT in a single visit. Agreement between RNFL thickness measurements was assessed using intraclass coefficient (ICC) and Bland-Altman plots. The discriminating abilities of the two techniques for detection of glaucoma were compared by the area under the receiver operating characteristic curves (AUC) for quadrants and average RNFL thickness. RESULTS: ICC values for agreement between both instruments were good for quadrants and average RNFL thickness (all ≥ 0.81). However, Spectralis OCT measurements were significantly greater than Cirrus OCT for temporal quadrant (difference = 4.27 µm in normal group, 3.91 µm in glaucoma group, p < 0.001 for both). The RNFL thickness parameter with the largest AUCs was the average RNFL thickness for the Spectralis OCT and the Cirrus OCT (0.85 vs. 0.87, p = 0.30). The pair-wise comparison among the receiver operating characteristic curves showed no statistical difference for all parameters. CONCLUSIONS: Although Spectralis OCT measurements were significantly greater than Cirrus OCT for temporal quadrant, agreement of RNFL measurement between both the devices was generally good and there was no statistically significant difference in the performance of glaucoma diagnosis between both instruments.
Area Under Curve ; Diagnosis ; Glaucoma ; Humans ; Nerve Fibers* ; Retinaldehyde* ; ROC Curve ; Tomography, Optical Coherence*

Acquired digital fibrokeratornas are uncommon, benign, acquired, firm, hyperkeratotic projectic projections, mostly arising at the finger, toe, and distal portion of extremity. We experienced a case of acquired digital fibrokeratoma arising the 4th tae in 37-year old male. Tumor mass was 2 X 1.5cm in size, round hyperkeratotic protruded mass. Histopathologically, the epidermis of the tumor showed compact hyperkeratosis, focal hypergranulosis, and irregular acanthosis. Thick collagen bundles and dilated capillaries were predominantly oriented in the direction of the longitudinal axis of the lesion. After simple excision was done, a new lesion appeared 2 months later.
Adult ; Axis, Cervical Vertebra ; Capillaries ; Collagen ; Epidermis ; Extremities ; Fingers ; Humans ; Male ; Toes

Celosia cristata , belongs to Amaranthaceae family, has been utilized in many traditional medicinal systems to treat hemostasis, eye and mouth inflammation, and gynecological diseases. The various physiological investigations on C. cristata have documented its antibacterial, antioxidant, antifungal, and antihepatotoxic properties. During the research program aimed at isolating bioactive constituents from the medicinal plants, the aerial parts of C. cristata were extracted using 80% EtOH, then sequentially partitioned with n-hexane, CH 2 Cl 2 , and EtOAc. The CH 2 Cl 2 -soluble fraction demonstrated inhibitory effects on nitric oxide production in LPS-induced RAW 264.7 cells, with an IC50 value of 24.7 μg/mL. The CH 2 Cl 2 -soluble fraction was subjected to a series of chromatographic techniques, such as Sephadex LH-20 column chromatography, MPLC, and preparative HPLC. As a result, seven known norisoprenoids (1–7) were isolated, and the structures were determined through the analysis of spectroscopic data, especially 1D NMR, 2D NMR, and HR-ESI-MS. Dehydrovomifoliol (2), 3-hydroxy-4,7-megastigmadien-9-one (6), and 9-hydroxy-4,7-megastigmadien-3-one (7) exhibited inhibitory effects on LPS-induced nitric oxide production in RAW 264.7 macrophages with IC50 values of 17.7–24.4 μM.

The aim of this study was to investigate the acute oral toxicity of fermented Scutellariae Radix (JKTMHGu-100) in rats and dogs. JKTM-HGu-100 was orally administered at a dose of 2,000 mg/kg in Sprague-Dawley rats. An escalating single-dose oral toxicity test in beagle dogs was performed at doses of 500, 1000, and 2000 mg/kg with 4-day intervals. Clinical signs, changes in body weight, mortality, and necropsy findings were examined for 2 weeks following oral administration. No toxicological changes related to the test substance nor mortality was observed after administration of a single oral dose of JKTM-HGu-100 in rats or dogs. Therefore, the approximate lethal dose (LD) for oral administration of JKTMHGu-100 in rats was considered to be over 2,000 mg/kg, and the maximum tolerance doses (MTDs) in rats and dogs were also estimated to be over 2,000 mg/kg. These results indicate that JKTM-HGu-100 shows no toxicity in rodents or non-rodents at doses of 2,000 mg/kg or less.
Administration, Oral ; Animals ; Body Weight ; Dogs ; Rats ; Rats, Sprague-Dawley ; Rodentia ; Scutellaria ; Scutellaria baicalensis ; Toxicity Tests

Eruptive vellus hair cysts(EVHCs) are small, red or brown colored cystic papules that usually occur on the chest and proximal extremities of children and adults. Although EVHCs, facial variant, is rare and chracteristic in its location and color, histopathological findings of facial variant are not different from those of typical EVHC. We report a case of EVHC, facial variant which occured on the face of 23-year-old man with asymptomatic, bluish papules. The lesions first appeared at the age of 19 years and had increased in number thereafter.
Adult ; Child ; Extremities ; Hair* ; Humans ; Thorax ; Young Adult