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J Y Lu

Publications and source records attributed to J Y Lu.

At least 19 recordsLinked to original sources
PubMed2003

Field intercomparison studies for evaluation and validation of the AESminiSamplR technique for sampling and analysis of total particulate mercury in the atmosphere.

This paper presents new data and reviews literature results from six field intercomparison studies to further evaluate and validate the AESminiSamplR [Anal. Chem., 70 (1998) 2403] technique for sampling and analysis of total particulate mercury in ambient air. The intercomparison studies were carried out at: two remote sites in the Arctic [Alert, Nunavut, Canada (82 degrees 28'N, 62 degrees 18'W) and Ny-Alesund, Svalbard (78 degrees 54'N, 11 degrees 53'E)], one site in Ann Arbor, MI, and three sites in Europe (one in Italy and two in Sweden). The AESminiSamplR was compared with five other methods using different materials, different flow rates, different sample treatment procedures, and/or different detection techniques. Four of the five methods compared were operated by different laboratories. When the compared methods were operated by different laboratories, a difference </=4 times was observed. When the compared methods were operated by the same laboratory, a maximum deviation of 1.3 times was achieved. These results demonstrate that the AESminiSamplR technique is a reliable, reproducible, simple, fast and cost-effective methodology for sampling and analysis of mercury associated with particulate matter in the atmosphere.

Air Pollutants↗
PubMed2001

Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis.

Deficiency in a recently characterized lysosomal enzyme, palmitoyl-protein thioesterase (PPT), leads to a severe neurodegenerative disorder of children, infantile neuronal ceroid lipofuscinosis (NCL). Over 36 different mutations in the PPT gene have been described, and missense mutations have been interpreted in the light of the recently solved X-ray crystallographic structure of PPT. In the current study, we assessed the biochemical impact of mutations through the study of cells derived from patients and from the expression of recombinant PPT enzymes in COS and Sf9 cells. All missense mutations associated with infantile NCL showed no residual enzyme activity, whereas mutations associated with late-onset phenotypes showed up to 2.15% residual activity. Two mutations increased the K(m) of the enzyme for palmitoylated substrates and were located in positions that would distort the palmitate-binding pocket. An initiator methionine mutation (ATG-->ATA) in two late-onset patients was expressed at a significant level in COS cells, suggesting that the ATA codon may be utilized to a clinically important extent in vivo. The most common PPT nonsense mutation, R151X, was associated with an absence of PPT mRNA. Mannose 6-phosphate modification of wild-type and mutant PPT enzymes was grossly normal at the level of the phosphotransferase reaction. However, mutant PPT enzymes did not bind to mannose 6-phosphate receptors in a blotting assay. This observation was related to the failure of the mutant expressed enzymes to gain access to 'uncovering enzyme' (N-acetylglucosamine-1-phosphodiester alpha-N-acetyl glucosaminidase), presumably due to a block in transit out of the endoplasmic reticulum, where mutant enzymes are degraded.

Age of Onset↗
PubMed2001

Positional candidate gene cloning of CLN1.

Mutations in the CLN1 gene encoding palmitoyl-protein thioesterase (PPT) underlie the recessive neurodegenerative disorder, infantile Batten disease, or infantile neuronal ceroid lipofuscinosis (INCL). The CLN1 gene was mapped to chromosome 1p32 in the vicinity of a microsatellite marker HY-TM1 in a cohort of Finnish INCL families, and mapping of the PPT gene to the CLN1 critical region (and the discovery of mutations in PPT in several unrelated families) led to conclusive identification of PPT as the disease gene. PPT is a lysosomal thioesterase that removes fatty acids from fatty-acylated cysteine residues in proteins. The accumulation of fatty acyl cysteine thioesters can be reversed in INCL cells by the exogenous administration of recombinant PPT, which enters the cells through the mannose 6-phosphate receptor pathway. Over two dozen PPT mutations have been found in PPT-deficient patients worldwide. In the United States, all PPT-deficient patients show "GROD" histology but the age of onset of symptoms is later in some children due to the presence of missense mutations that result in enzymes with residual PPT activity. Now that INCL is known to be caused by a defect in a soluble lysosomal enzyme, appropriate therapies may be forthcoming. Prospects for therapy include enzyme replacement, stem cell transplantation, gene therapy, and metabolic therapy aimed at depleting the abnormal substrate accumulation in the disease.

Cloning, Molecular↗
PubMed2001

Infantile neuronal ceroid lipofuscinosis: no longer just a 'Finnish' disease.

The neuronal ceroid lipofuscinoses (NCLs) are a group of enigmatic neurodegenerative disorders of children that have in common the storage of autofluorescent lipofuscin, or aging pigment, in the brain. With the identification of the three major genes involved in the disorder, the NCLs are now appreciated to represent true lysosomal storage disorders. The most severe (infantile) form of NCL is caused by mutations in a lysosomal thioesterase that removes fatty acids from modified cysteine residues in proteins. Although the disorder was first described in Finland (and the identification of the underlying gene (CLN1) made in this population) defects in CLN1 and the underlying deficiency have been widely reported outside of Scandinavia. In this report, we summarize the relationship of genotype to phenotype in the disorder and evaluate known mutations in light of the recently solved crystal structure of defective enzyme, palmitoyl-protein thioesterase (PPT). We also discuss progress in identifying the fatty acyl cysteine thioesters that accumulate in PPT deficiency and in working toward animal models of NCL. Recent progress in these areas holds promise for the eventual treatment of the disorder.

Animals↗
PubMed2001

Multiple pleural nodules without effusion--a rare presentation of tuberculous pleurisy.

We report a rare case of tuberculous pleurisy presenting with multiple pleural nodules without associated effusion or parenchymal lung lesions. A 62-year-old man had multiple discrete pleural nodules in the right hemithorax on chest radiography without any clinical symptoms. Thoracoscopic biopsy of the pleural nodules revealed a caseous granuloma with acid-fast bacilli. The patient received antituberculous therapy, with resolution of tuberculomas on chest film within 2 months. To our knowledge, only two similar cases have been previously reported in the English literature, and our observation should lead to broadening of the spectrum of the differential diagnosis of multiple pleural nodules.

Humans↗
PubMed2001

Diabetic ketoacidosis and hypogonadotropic hypogonadism in association with transfusional hemochromatosis in a man with beta-thalassemia major.

We report a 23-year-old man with beta-thalassemia major and transfusional hemochromatosis, which manifested as diabetic ketoacidosis and hypogonadotropic hypogonadism. This unusual presentation of diabetic ketoacidosis in hemochromatosis has rarely been reported. Magnetic resonance imaging of the abdomen showed decreased signal intensity in the liver, spleen, and pancreas. In addition, the pituitary gland also showed heterogeneous low signal intensity, compatible with hemochromatosis. He was treated with insulin supplements and pulsatile human chorionic gonadotropin administration. Clinical improvement was noted after hormone replacement. Intensive iron chelation therapy was given to prevent cardiac complications, and to restore his gonadal function. During follow-up, the patient experienced improvement in libido and sexual potency.

Adult↗
PubMed2001

Diagnostic pitfalls of fine-needle aspiration cytology and prognostic impact of chemotherapy in thyroid lymphoma.

BACKGROUND AND PURPOSE: Fine-needle aspiration cytology (FNAC) is an important method in the evaluation of goiter. However, difficulties are encountered when using this technique to distinguish Hashimoto's thyroiditis from thyroid lymphoma. This study sought to determine the diagnostic sensitivity of FNAC and to determine the effectiveness of chemotherapy in the treatment of thyroid lymphoma. METHODS: We retrospectively reviewed the clinical manifestations, diagnostic methods, treatment, and prognosis in 14 consecutive patients with histopathology-verified thyroid lymphoma treated in National Taiwan University Hospital from 1981 to 2000. RESULTS: Eleven of the 14 patients underwent FNAC, which identified six lymphomas, one anaplastic carcinoma, three cases of Hashimoto's thyroiditis, and one case of Riedel's struma. Because all cases were promptly biopsied, the mean survival times for patients with or without the correct initial diagnosis (15 mo vs 43 mo) did not differ significantly (p = 0.098 by Student's t-test). Thyroid lymphoma was diagnosed before 1990 in four patients, three of whom were treated with local radiotherapy and one with surgical resection. The mean survival time of these four patients was 60 days. Thyroid lymphoma was diagnosed after 1990 in 10 patients, nine of whom underwent systemic chemotherapy, with additional adjunctive radiotherapy in three patients. The mean survival time in the nine of these 10 patients with follow-up was 60 months. A significant difference was found in the mean disease-free survival times between patients treated before and after 1990 (60 d vs 60 mo, p = 0.005). CONCLUSIONS: In this study, FNAC had a sensitivity of only 55%, its major limitation being misdiagnosis of lymphoma as Hashimoto's thyroiditis in three patients. However, such initial misdiagnosis does not affect the prognosis if promptly corrected by histopathology. As evidenced by the improved survival of patients receiving chemotherapy after 1990, we conclude that chemotherapy is effective in the treatment of thyroid lymphoma.

Adult↗
PubMed2001

Protective effects of tea polyphenols on myocardial free radical metabolic disorder in mice induced by inhalation of pure oxygen under 5500 m hypobaric condition.

OBJECTIVE: To observe the protective effects of natural antioxidant tea polyphenols(TP) on myocardial free radical metabolic disorder in mice induced by inhalation of hypobaric pure oxygen under 5500 m hypobaric condition. METHOD: Forty-two male Kunming mice were randomly divided into three groups (n = 14 each): group A, normal control; group B, inhalation of pure oxygen (> 96 %) at simulated altitude of 5500 m in an animal altitude chamber; group C (TP protection group), same as group B but 100 mg/kg of TP was given orally before the exposure. The exposure time was 2 h/d, 3 d/wk for a total of 8 wk, and distilled water was given to groups A and B before exposure. After experiment, the mice were decapitated on the next day and the heart was quickly removed. Malondialdehyde (MDA) concentration, superoxide dismutase (SOD) activity and nitric oxide (NO) content were measured. In addition, Cu, Zn-SOD and inducible NO synthase (iNOS) enzymatic contents in myocardial tissue were qualitatively examined by immunohistochemical assaying. RESULT: Compared with the control, MDA concentration, SOD activity and Cu, Zn-SOD enzymatic content in group B were significantly increased (P < 0. 05). But in TP protection group, myocardial MDA formation was significantly decreased (P < 0. 01) and SOD activity and Cu, Zn-SOD expression restored to normal. On the contrary, myocardial NO generation and iNOS expression were significantly reduced after repeated inhalation of hypobaric oxygen at 5500 m. NO metabolism regained to normal after repeated administration of TP. CONCLUSION: Natural antioxidant TP had protective effects on myocardial free radical metabolic disorder induced by inhalation of hypobaric pure oxygen under 5500 m hypobaric condition.

Altitude↗
PubMed2001

Protective effects of tea polyphenols on mild hypobaric hypoxia induced pulmonary free radical metabolic disorder in mice.

Objective. To observe the protective effects of natural antioxidant tea polyphenols (TP) on repeated mild hypobaric hypoxia induced pulmonary free radical metabolic disorder in mice. Method. Fourty-two male Kunming mice were randomly divided into three groups (n=14 each): normal control (A); 1500 in mild hypobaric hypoxia (B) and TP protection group (C). The exposure time in hypobaric chamber was 2 h/d, 3d/wk, 8 wk in total. Before hypoxic exposure, TP was orally given to group C at a dose of 100 mg/kg, while distilled water was given to the other two groups. After experiment, the mice were decapitated on the next day and the lung was quickly removed. The malondialdehyde (MDA) concentration, superoxide dismutase (SOD) activity and nitric oxide (NO) content were measured. In addition, Cu, Zn-SOD and inducible NO synthase (iNOS) enzymatic contents in lung were were qualitatively examined by immunohistochemical assaying. Result. Compared with the control group, pulmonary MDA concentration and NO content were significantly increased after chronic mild hypobaric hypoxic exposures (P<0.01) but the MDA formation and NO generation in TP protection group were restored to normal. Pulmonary SOD activity in group B tended to increase. Cu, Zn-SOD expression in endothelial cells of bronchioli and iNOS contents in endothelial cells of bronchioli and endothelial cells and smooth muscle cells in pulmonary interstitial vessels were significantly elevated after repeated mild hypobaric hypoxic exposure. These enzymatic abnormal expressions regained to normal after administration of TP. Conclusion. Natural antioxidant TP had protective effects on repeated mild hypobaric hypoxia induced pulmonary free radical metabolic disorder.

Altitude↗
PubMed2001

[Effects of repeated high +Gz exposure on several enzyme activities in cardiomyocytes in rats and some protective measures].

Objective. To determine the changes of several myocardial enzymes in rats after repeated high +Gz exposure and the protective effects of preconditioning of low-G exposure and tea polyphenols (TP). Method. Thirty-two male Wistar rats were randomly divided into 4 groups (n = 8 each): control group (group A), +10 Gz group (group B), low-G preconditioning group (group C) and TP protection group (group D). Group B, C and D were exposed to repeated +10 Gz stress (each for 30s, 5 times/d with +1 Gz 1 min intervals, 3 d/wk, 3 weeks in total), but group A was only submitted to +1 Gz for 5 min. Group C was exposed to +2 Gz for 5 min about 1 h prior to +10 Gz stress. Additionally, TP (200 mg/kg) was given orally to group D about 1 h prior to the +Gz experiment, while distilled water was given to groups A, B and C instead. On the next day after the last centrifuge run, the hearts were taken out immediately for making frozen tissue sections. Enzyme histochemical staining and image analysis were carried out for acid phosphatase (ACP), succinate dehydrogenase (SDH), cytochromeoxidase (Cyt aa3) and adenosine triphosphatase (ATPase). Result. As compared with the control, the activities of ACP and SDH in +10 Gz stressed rats decreased significantly (P<0.05), and there was a declining trend for Cyt aa3. But, low-G preconditioning and TP had protective effects on +10 Gz stress-induced reduction of these enzymatic activities (P<0.05). Conclusion. The results showed that repeated high +Gz exposure could bring about decreases of activities in ACP (the marker enzyme of lysosome) and SDH (the marker enzyme of mitochondrial endomembrane), which indicated a reduction of oxidative metabolism in myocardial tissue; but preconditioning with low-G and natural antioxidant TP had protective effects.

Acceleration↗
PubMed2001

[Inhibition of Na+/Ca2+ exchange by tetrapeptide FMRFa in intact rat ventricular myocytes].

AIM: To study the effects of Phe-Met-Arg-Phe-NH2(FMRFa) on Na+/Ca2+ exchange and its specificity for Na+/Ca2+ exchange in rat ventricular myocytes. METHODS: Na+/Ca2+ exchange current and other currents of ion channels were measured using whole cell voltage clamp techniques. RESULTS: A dose-related inhibition of tetrapeptide FMRFa on Na+/Ca2+ exchange was observed in rat ventricular myocytes. Inward and outward INa+/Ca2+ were inhibited by 60.1% and 56.5%, respectively, at highest concentration (100 mumol.L-1) and its IC50 were 20 mumol.L-1 and 34 mumol.L-1 in inward and outward INa+/Ca2+, respectively. Inward and outward INa+/Ca2+ were inhibited 38.7% and 34.9%, respectively, at FMRFa 5 mumol.L-1. FMRFa 5 mumol.L-1 and 20 mumol.L-1 did not affect L-type calcium current, sodium current, transient outward current and inward rectifier potassium current. CONCLUSION: These data indicate that FMRFa is a specific inhibitor of Na+/Ca2+ exchange in intact rat ventricular myocytes.

Animals↗
PubMed2000

The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis.

Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the formation of neuronal storage bodies that are associated with the clinical symptoms. Less severe forms of PPT1 deficiency have been found recently that are caused by a distinct set of PPT1 mutations, some of which retain a small amount of thioesterase activity. We have determined the crystal structure of PPT1 with and without bound palmitate by using multiwavelength anomalous diffraction phasing. The structure reveals an alpha/beta-hydrolase fold with a catalytic triad composed of Ser115-His289-Asp233 and provides insights into the structural basis for the phenotypes associated with PPT1 mutations.

Amino Acid Sequence↗
PubMed2000

Effects of phase aberration on high frame rate imaging.

A high frame-rate (HFR) imaging method (about 3750 frames/s for imaging of biological soft tissues at a depth of 200 mm) has been developed recently with limited diffraction beams. This method uses the fast Fourier transform (FFT) and inverse fast Fourier transform (IFFT) to construct images, and can be implemented with simple and inexpensive hardware, compared to the conventional delay-and-sum method where a digital beam former is usually used. In this paper, phase aberration effects are studied for both the high frame rate and the conventional methods by adding random phase shifts to echo signals obtained from an experiment. In the study, two broadband linear arrays were used to construct images of an ATS 539 tissue-equivalent phantom that has a frequency-dependent attenuation of about 0.5 dB/MHz/cm. The first array has 48 elements, a central frequency of 2.25 MHz, an aperture of 18.288 mm, and a width of 12.192 mm in elevation. The second has 64 elements, a central frequency of 2.5 MHz, and a dimension of 38.4 mm x 10 mm. The-6dB pulse-echo bandwidth of both arrays is about 40% of their center frequencies. Radiofrequency (RF) signals were digitized at 20 mega samples/s at a 12-bit resolution to construct images. Results show that phase aberration has about the same effect on both methods in terms of image resolution and contrast, although the high frame-rate method can be implemented with a simpler system.

Image Processing, Computer-Assisted↗
PubMed2000

Polyclonal anticardiolipin antibodies purified from sera of patients with active systemic lupus erythematosus induce apoptosis of the cultured glomerular mesangial cells.

OBJECTIVE: To test the effect of anticardiolipin antibodies (aCL) on cultured glomerular mesangial cells with regard to their expression of apoptosis-related genes. METHODS: aCL purified from active lupus sera by cardiolipin micelles were incubated with cultured rodent mesangial cells (RMC). Morphological changes of the RMC were observed. The genomic DNA was extracted for the detection of apoptosis. The total cell RNA was extracted for detection of Fas, c-myc, p53, and bcl-2 transcripts by reverse transcription-polymerase chain reaction. RESULTS: aCL (100 GPL-U/0.1 mg protein/ml) bound to RMC more prominent than human IgG (100 microg/ml). The antibodies suppressed RMC proliferation in a dose-dependent manner. The RMC were undergoing apoptosis as evidenced by morphologic changes, fluoresceinannexin V staining and appearance of nucleosome-sized DNA fragments. RMC spontaneously express p53 and c-myc but not Fas or bcl-2. aCL (100 GPL-U/ml) enhanced the expression of Fas but not other apoptosis-related genes and suppressed the intracellular tyrosine phosphorylation. CONCLUSIONS: Binding of aCL can induce apoptosis of the RMC. The aCL may be implicated in the pathogenesis of lupus nephritis.

Animals↗
PubMed2000

Second-harmonic generation of the nth-order Bessel beam.

We investigate the second-harmonic generation of the nth-order Bessel beam in the nonlinear medium. The analysis is based on the Khokhlov-Zabolotskaya-Kuznetsov wave equation under the second-order approximation in nonlinear acoustics. The theory indicates that for an nth-order Bessel beam, the second-harmonic beam is nearly diffraction-free in the radial direction and behaves as a Bessel beam of the order 2n, and that the axial pressure amplitude is proportional to the square root of propagation distance. A variety of applications in many fields of nonlinear acoustics and nonlinear optics is expected.

Acoustics↗