• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.14-22
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• 2012

Objectives: This study was performed to review clinical manifestations of hyperphenylalaninemia patients in Korean. Methods: 178 cases of hyperphenyalaninemia were diagnosed at department of pediatrics, Soonchunhyang University Hospital from January, 1983 to August. We reviewed DNA analysis, MR imaging, EEG, radiography of the left hand and wrist, bone densitometry (BMD), IQ test of hyperphenyalaninemia patients. Results: Out of 178 cases, 161 cases were diagnosed classic phenylketonuria and 17 cases were diagnosed BH4 deficiency. 122 cases performed DNA analysis. R243Q (10.3%), Y204C (9.9%), and IVS4-1G>A (8.1%) mutations were predominant. 22 cases underwent MR imaging. Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the periventricular deep white matter of 15 cases. 23 cases were performed EEG. 12 cases (52.3%) showed abnormal pattern. EEG abnormalities showed in 11 cases. On lumbar BMD four of 11 cases (36%) showed reduced bone density of more than 1 S.D. in four of 11 cases, bone age was less than chronological age by at least one year. 18 cases were performed IQ test. Mean IQ scores was $84{\pm}21.6$. Among older than 15 years (9 cases), Mean IQ scores was $72{\pm}21.2$. PTPS deficiency was 14 cases, DHPR deficiency was 2 cases, and GTPCH deficiency was 1 case. Conclusion: We confirmed there were varieties of DNA mutations. And MR imaging and EEG were nonspecific in PKU patients. Older children showed lower IQ score. Low phenylalanine diet prevents brain damage in PKU patient. Not only first few years of life but also lifetime, Keeping low phenylalanine diet is important.

• Journal of Life Science
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• v.27 no.11
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• pp.1369-1375
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• 2017

The purpose of this study was to investigate the cellular characterization of phospholipase C-${\delta}1$ in olive flounders (Paralichthys olivaceus). In general, phospholipase C signaling pathways are distributed in nuclei at plasma membranes and in cytoplasms, although the pathways' nuclear localization mechanisms are unclear. P. olivaceus duplicates type-A PoPLC-${\delta}1$ (PoPLC-${\delta}1A$), which has a high similarity to the human isoform PLC-${\delta}$; type-B PoPLC-${\delta}1$ (PoPLC-${\delta}1B$ [Sf]), which has a low similarity to the human isoform PLC-${\delta}$ and the alternative splice variant PoPLC-${\delta}1B$ (Lf), which has a nuclear localization signal (NLS) and a nuclear export signal (NES) for nuclear imports and exports, respectively. This study confirmed the effects of the cellular localization and translocation of GFP-tagged PoPLC-${\delta}1A$, PoPLC-${\delta}1B$ (Sf) and PoPLC-${\delta}1B$ (Lf). It administered treatments of $Ca^{2+}$ ionophore ionomycin and endoplasmic reticulum (ER)-$Ca^{2+}$ pump inhibitor thapsigargin to hirame natural-embryo (HINAE) cells. A laser-scanning confocal microscope was used. GFP-tagged PoPLC-${\delta}1A$ was distributed to the cellular organelles, rather than to the cytoplasms and cytomembranes, when PoPLC-${\delta}1B$ (Lf) and PoPLC-${\delta}1B$ (Sf) were localized at the plasma membranes. The treatments of ionomycin and thapsigargin showed the accumulation of PoPLC-${\delta}1A$ in the nuclei when PoPLC-${\delta}1B$ (Lf) nucleocytoplasmic shuttling and PoPLC-${\delta}1B$ (Sf) nucleocytoplasmic shuttling were not observed. The results were the first evidence that PoPLC-${\delta}1A$, which contains functional, intact NES sequences, has a main role in nucleocytoplasmic shuttling and translocation in fish.

• Journal of the Korean Vacuum Society
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• v.19 no.3
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• pp.211-216
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• 2010

The luminescence properties of $In_{0.5}Ga_{0.5}As/In_{0.5}Al_{0.5}As$ multiple quantum wells (MQWs) grown on $In_{0.5}Al_{0.5}As$ buffer layers have been studied by using photoluminescence (PL) and time-resolved PL measurements. A$1-{\mu}m$ thick $In_{0.5}Al_{0.5}As$ buffer layers were deposited on a 500 nm thick GaAs layer, followed by the deposition of the InGaAs/InAlAs MQWs. In order to investigate the effects of InAlAs buffer layer on the optical properties of the MQWs, four different temperature sequences are used for the growth of InAlAs buffer layer. The growth temperature for InAlAs buffer layer was varied from 320^{\circ}C to $580^{\circ}C$. The MQWs consist of three $In_{0.5}Ga_{0.5}$As wells with different well thicknesses (2.5 nm, 4.0 nm, and 6.0 nm thick) and 10 nm thick $In_{0.5}Al_{0.5}$As barriers. The PL spectra from the MQWs with InAlAs layer grown at lower temperature range ($320-580^{\circ}C$) showed strong peaks from 4 nm QW and 6 nm QW. However, for the MQWs with InAlAs buffer grown at higher temperature range ($320-480^{\circ}C$), the PL spectra only showed a strong peak from 6 nm QW. The strongest PL intensity was obtained from the MQWs with InAlAs layer grown at the fixed temperature of $480^{\circ}C$, while the MQWs with buffer layer grown at higher temperature from $530^{\circ}C$ to $580^{\circ}C$ showed the weakest PL intensity. From the emission wavelength dependence of PL decay times, the fast and slow decay times may be related to the recombination of carriers in the 4 nm QW and 6 nm QW, respectively. These results indicated that the growth temperatures of InAlAs layer affect the structural and optical properties of the MQWs.

• Journal of the Korean Vacuum Society
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• v.20 no.6
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• pp.449-455
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• 2011

The luminescence properties of $In_{0.5}Ga_{0.5}As/In_{0.5}Al_{0.5}As$ multiple quantum wells (MQWs) grown on $In_{0.4}Al_{0.6}As$ buffer layer have been investigated by using photoluminescence (PL) and time-resolved PL measurements. A 1-${\mu}m$-thick $In_{0.4}Al_{0.6}As$ buffer layers were deposited at various temperatures from $320^{\circ}C$ to $580^{\circ}C$ on a 500-nm-thick GaAs layer, and then 1-${\mu}m$-thick $In_{0.5}Al_{0.5}As$ layers were deposited at $480^{\circ}C$, followed by the deposition of the InGaAs/InAlAs MQWs. In order to study the effects of $In_{0.4}Al_{0.6}As$ layer on the optical properties of the MQWs, four different temperature sequences are used for the growth of $In_{0.4}Al_{0.6}As$ buffer layer. The MQWs consist of three $In_{0.5}Al_{0.5}As$ wells with different well thicknesses (2.5-nm, 4.0-nm, and 6.0-nm-thick) and 10-nm-thick $In_{0.5}Al_{0.5}As$ barriers. The PL peaks from 4-nm QW and 6-nm QW were observed. However, for the MQWs on the $In_{0.4}Al_{0.6}As$ layer grown by using the largest growth temperature variation (320-$580^{\circ}C$), the PL spectrum only showed a PL peak from 6-nm QW. The carrier decay times in the 4-nm QW and 6-nm QW were measured from the emission wavelength dependence of PL decay. These results indicated that the growth temperatures of $In_{0.4}Al_{0.6}As$ layer affect the optical properties of the MQWs.

• Journal of Marine Life Science
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• v.8 no.2
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• pp.104-114
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• 2023

As plastic usage increases globally, the amount of plastic waste entering the marine environment is steadily rising. Microplastics, in particular, can be ingested by marine organisms and accumulated in their digestive tracts, causing harmful effects on their growth and reproduction. Cytochrome P450 (CYP) enzymes are known to metabolize various environmental pollutants as detoxification enzymes, but their role in crustaceans is not well understood. In this study, sequences of nine CYP genes (CYP370A4, CYP370C5 from clan 2; CYP350A1, CYP350C5, CYP361A1 from clan 3; CYP4AN-like, CYP4AP2, CYP4AP3, CYP4C33-like1 from clan 4) were analyzed using conserved domains in the brackish water flea Diaphanosoma celebensis. Additionally, after exposure to three different sizes of polystyrene beads (0.05-, 0.5-, 6-㎛ PS beads; 0.1, 1, and 10 mg/L) for 48 hours, the expression of these nine CYP genes were investigated using real-time reverse transcription polymerase chain reaction (RT-PCR). The results showed that all CYP genes possessed conserved motifs, indicating that D. celebensis CYP has evolutionarily conserved functions. Among these CYP genes, the expression of CYP370C5, CYP360A1, and CYP4C122 showed a significant increase after exposure to 0.05-㎛ PS beads, suggesting their involvement in PS metabolism. This research will contribute to understanding the molecular mode of actions of microplastics on marine invertebrates.