PURPOSES: Regional myocardial functional parameters were assessed by ECG-gated myocardial SPECT analysis in normal subjects and ischemic heart diseases. METHODS: Normal subjects (13 male and 10 female) and 51 patients with chronic ischemic heart disease underwent ECG-gated myocardial SPECT. A dose of 740 MBq of 99mTc-MIBI was injected at rest, and gated SPECT was performed 60 min later. Wall motion (WM) and systolic wall thickening (WT), % tracer uptake were evaluated by quantitative gated SPECT program (QGS). Regional parameters were obtained in the 16 segments based on the functional polar map. In the normal group, standard values were evaluated in the lateral, septal, anterior and inferior regions both in male and female subjects. In the ischemic heart disease group, sensitivity and specificity of these parameters were assessed in each segment. To estimate the ability of WM and WT in detecting regional dysfunction, decreased perfusion area, which was defined as < mean - 2SD by the normal profile, was used as a standard. The receiver operating characteristics (ROC) area analysis was also performed. RESULT: In the normal profile, % tracer uptake was decreased in the anterior segments of female group, no significant difference was observed between male and female in WM and WT. WM was decreased in the septum and increased in the lateral segment. WT didn't show any difference regional difference. Sensitivity and specificity of WM were 56%/91% in the anterior, 0%/100% in the septum, 43%/87% in the inferior, 31%/85% in the lateral. WT were 67%/93% in the septum, 67%/79% in the inferior, and 59%/81% in the septum. The area under ROC curve was WM 0.63, WT 0.85 (p < 0.005 between WM and WT) in the septum, in inferior WM 0.77, WT 0.80 (p = 0.57), in anterior WM 0.86, WT 0.87 (p = 0.095), in lateral WM 0.68, WT 0.78 (p = 0.037). CONCLUSION: In normal profile, the % tracer uptake in the anterior wall decreased in females, but WM and WT did not show significant difference in each region affected this influence. The septal WM was decreased in the normal profile and ability to diagnose regional function was also decreased compared with WT. Thus, we can conclude that WT is preferable for detecting septal functional abnormality.

Aged↗

PubMed1999

Dentatorubral-pallidoluysian atrophy (DRPLA): clinical features and molecular genetics.

Explore the source record for details and available documents.

Atrophy↗

PubMed1999

Age-related changes in the cerebral distribution of 99mTc-ECD from infancy to adulthood.

UNLABELLED: Although cerebral blood flow in infants differs from that in older individuals, the distribution of 99mTc-ethyl cysteinate dimer (ECD) in infants has not been well studied. This study compared 99mTc-ECD distribution in infants and children with that in young adults. METHODS: 99mTc-ECD SPECT was performed on 37 patients suspected of having epilepsy, ranging in age from 3 mo to 26 y. The patients were divided into two age-matched groups, a drug-free group (n = 19) and a drug-taking group (n = 18), according to their anticonvulsant medication status at the time of examination. 99mTc-ECD (100-740 MBq) was injected interictally, and SPECT data were acquired using a triple-head gamma camera. Mean whole-brain counts were obtained from 10 sequential SPECT images. Regions of interest were set bilaterally on five areas of the cerebral cortex and on the basal ganglia, thalamus and cerebellum. The brain perfusion index (BPI) was obtained as a ratio of the mean counts in each region of interest to the mean whole-brain counts. The relationship between BPI and age in each region in the drug-free and drug-taking groups was analyzed separately and together using linear regression. The relationship between five patient age groups (<1 y, n = 4; 1-4 y, n = 9; 5-9 y, n = 8; 10-15 y, n = 7; >15 y, n = 9) and BPI in each region was also examined using multiple comparison analyses. RESULTS: Significant positive correlations between BPI and age in the frontal cortex and cerebellum were confirmed in the drug-free group. Anticonvulsant drugs did not affect the regression lines of BPI in the frontal cortex and cerebellum. Significant differences in BPI between age groups were seen in the parietal cortex, frontal cortex, occipital cortex, basal ganglia, thalamus and cerebellum in all patients. CONCLUSION: Age-related changes in cerebral 99mTc-ECD distribution were confirmed and found to be unaffected by the administration of anticonvulsant drugs. 99mTc-ECD uptake in children and infants is different from cerebral blood flow glucose metabolism as previously reported, especially in the cerebellum.

Adolescent↗

PubMed1999

[An early-onset case of acute disseminated encephalomyelitis with bilateral thalamic lesions on MRI].

We report the case of 5-year-old girl with acute disseminated encephalomyelitis (ADEM), whose MRI showed bilateral thalamic lesions. She suffered from left optic neuritis and generalized convulsion. Examination of cerebrospinal fluid revealed elevation of mononuclear cells and myelin basic protein (MBP). MRI showed the swelling of left optic nerve and high intensity areas of bilateral thalamus. After methylprednisolone pulse therapy, her visual acuity was dramatically improved and bilateral thalamic lesions were decreased. In childhood, bilateral thalamic lesions were observed in several diseases, such as viral encephalitis. Reye syndrome, Leigh syndrome and acute necrotizing encephalopathy. Demyelinating diseases involving the grey matter were very rare, but we must consider the presence of symmetrical thalamic involvement in patients with ADEM.

Child, Preschool↗

PubMed1999

[A case of post-irradiation lumbosacral radiculopathy successfully treated with corticosteroid and warfarin].

A 33-year-old man underwent post-operative radiation therapy for the left testicular anaplastic seminoma. One year later, the patient developed muscle weakness and sensory disturbance in the left lower extremity, and muscle weakness in the right lower extremity. MRI demonstrated linear and focal gadolinium (Gd) enhancement of the anterior portion of the lumbosacral roots within the cauda equina. The neurological symptoms improved after administration of corticosteroid and warfarin. Radiation myelopathy of this type was classified as "selective anterior horn cell injury or amyotrophy" by Reagan, and the site of the lesion was considered to be the lower motor neurons. However, based on the clinical and MRI findings, we proposed that the disease process was injury to the spinal nerve roots rather than the lower motor neurons. Recent neuropathological studies of this syndrome have demonstrated degeneration of the proximal spinal nerve roots. We consider that primary lesions of this syndrome occur in spinal nerve roots rather than in lower motor neurons, and "lumbosacral radiculopathy" is a more appropriate term for this condition.

Adult↗

PubMed1999

Ultrastructural evidence for dendritic release of acetylcholinesterase in the rat substantia nigra.

Morphological evidence for dendritic secretion of acetylcholinesterase (AChE) in rat substantia nigra--a physiologically known phenomenon--was searched by means of a modified cytochemical method devised for fine localization of AChE activity at the electron microscopic level. DAB precipitate was observed in cluster of small vesicles in contact with the plasma membrane and in the extracellular space in the vicinity of the vesicles. Single coated or uncoated large vesicles filled with stained material were found in the cytoplasm of the dendrites at distance from or in contact with the plasma membrane. Immunoperoxidase staining with specific anti-serum against rat AChE gave similar localization of AChE. These results suggest that AChE is released from the dendrites of the nigral neurons by a process of vesicular exocytosis and captured by endocytosis. The relation of this process to a putative release from the smooth endoplasmic reticulum remains to be elucidated.

Acetylcholinesterase↗

PubMed1999

Cyclooxygenase inhibitors suppress angiogenesis and reduce tumor growth in vivo.

Angiogenesis plays a key role in the development of malignant tumors. To clarify the roles of cyclooxygenase (COX) in malignant tumor development and angiogenesis, we investigated the effects of COX inhibitors on two kinds of gastrointestinal cancer xenograft, one of which overexpresses COX-2 and the other expresses no COX, in nude mice in vivo. There was a positive correlation between tumor volume and angiogenesis within the xenograft. Oral administration with a specific COX-2 or a nonspecific COX inhibitors lowered the expression of potent angiogenic factors; vascular endothelial growth factor and basic fibroblast growth factor, reduced angiogenesis and growth, induced apoptosis, and suppressed cell replication of the COX-2 overexpressing cancer xenografts in a dose-dependent manner. A nonspecific COX inhibitor, not a specific COX-2 inhibitor, reduced growth and angiogenesis of non-COX expressing cancer xenograft by inhibition of COX-1 in vascular endothelial cells. These results demonstrate that COX inhibitors suppress angiogenesis and tumor growth by inhibiting expression of angiogenic factors and vascular endothelial cell growth. They support the hypothesis that COX plays an important role in cancer growth via angiogenesis. These findings offer a new strategy against cancer using COX inhibitors (nonsteroidal anti-inflammatory drugs).

Animals↗

PubMed1999

Shear-dependent functions of the interaction between soluble von Willebrand factor and platelet glycoprotein Ib in mural thrombus formation on a collagen surface.

Recent flow studies have clearly established the function of von Willebrand factor (vWF) in initial platelet adhesion, in which the interaction of surface-immobilized vWF with platelet glycoprotein (GP) Ib, particularly at high shear rates, leads to the transient capture of flowing platelets onto the surface. This interaction is thought to trigger activation of platelet GP IIb/IIIa, leading to irreversible platelet adhesion and subsequent mural thrombus growth. The role of vWF-GP Ib interaction in secondary thrombus growth remains to be clarified, however. In this study, time-course images of the thrombus formation process were obtained using a whole blood flow system that allows real-time visualization of fluorescence-labeled platelet thrombus formation. The system employs a collagen-coated surface in a parallel plate flow chamber mounted on an epifluorescence microscope, which is then subjected to computer-assisted image analysis. In perfusion of blood preincubated with anti-vWF antibody NMC-4, which blocks the vWF-GP Ib interaction in solution, neither primary platelet adhesion nor subsequent thrombus growth on a collagen surface was detected at high shear rates (> or = 1210/s). In addition, even under experimental conditions in which initial platelet adhesion normally occurred, NMC-4-treated blood perfusion showed an apparent defect of secondary thrombus growth at the same high shear rates. The overall process of thrombus formation at low shear rates (< or = 340/s) was not affected by specific blockers of the vWF-GP Ib interaction. These findings indicate that, in addition to the interaction of surface-immobilized vWF with GP Ib in platelet adhesion, the interaction of soluble vWF with GP Ib is required for secondary thrombus growth selectively at high shear rates.

Antibodies, Monoclonal↗

PubMed1999

[Assessment of regional quantitative analysis by ECG-gated myocardial SPECT after coronary artery bypass surgery].

PURPOSE: ECG-gated myocardial SPECT (G-SPECT) was performed before and after coronary artery bypass surgery (CABG) to investigate how this operation would affect the assessment of regional quantitative analyses. METHODS: Nineteen patients with coronary artery disease underwent G-SPECT before and 1 month after uncomplicated CABG. 99mTc-MIBI 740 MBq was injected at rest, then G-SPECT was performed 60 min later. Regional ejection fraction (rEF), wall motion (WM), systolic wall thickening (WT) and % tracer uptake were evaluated by quantitative gated SPECT program (QGS). Parameters were obtained quantitatively in 16 segments based on the functional bull's eye map. RESULTS: Percent tracer uptake increased in septum from 75 +/- 11% to 78 +/- 11% (p < 0.001), while WT did not change (40 +/- 19% to 41 +/- 20%) after CABG. However, in septum rEF decreased from 17 +/- 13% to 6 +/- 9% (p < 0.001) and WM decreased in septum from 1.6 +/- 1.1 mm to 0.6 +/- 0.9 mm (p < 0.001). CONCLUSION: Significant reduction of rEF and WM despite of no deterioration of WT and % tracer uptake suggested that rEF and WM were affected by pseudoparadoxical asynergy after uncomplicated CABG. For the evaluation of regional function after CABG by G-SPECT, WT might be the preferred parameter.

Aged↗

PubMed1999

[Familial amyotrophic lateral sclerosis associated with mutant Cu/Zn superoxide dismutase as a conformational disease].

To investigate the molecular mechanism of mutant Cu/Zn superoxide dismutase (SOD) associated with familial amyotrophic lateral sclerosis (FALS), mutant (A1a4Thr, Gly85Arg, Gly93Ala, and two base-pair deletion in the 126th codon), as well as wild-type (wt), Cu/Zn SODs were expressed in COS7 cells. The formation of granular cytoplasmic aggregates accompanied by collapse of the cytoplasm was observed in cells expressing mutant Cu/Zn SODs, but not in cells expressing mutant Cu/Zn SODs. The aggregates contained ribosome-like particles and endoplasmic reticulum. These results suggest the possibility that mutant Cu/Zn SODs promote the formation of aggregates which are toxic to cells.

Amyotrophic Lateral Sclerosis↗

PubMed1999

[Studies on rejected food yellow No. 5 (sunset yellow FCF)].

One of the sunset yellow FCFs (Y-5) in the official inspection of coal-tar dyes in 1998 was rejected. The results of tests of the rejected sample were submitted to JSFA-VI except for a sub-spot by paper chromatography. HPLC of the raw materials, intermediates, and subsidiary dyes according to JSFA-VII was performed on the rejected and submitted samples of Y-5. The sub-spot in the rejected sample was identified as sulfanilic acid-azo-R salt, and its content was estimated at more than 5%.

Chromatography, High Pressure Liquid↗

PubMed1999

[Estimated production by the official inspection of coal-tar dyes (including dye aluminum lakes) in fiscal year 1998].

There were 284 official inspections of coal-tar and their lakes in fiscal year 1998, and 283 of the lots qualified, one of the 40 samples of Food Yellow No. 5 was rejected. The quality of coal-tar dyes that passed inspection in Japan in fiscal year 1998 reached 150.3 tons. Coal-tar dye production is summarized by manoth in Table 2 and by manufacturer in Table 3. The food coal-tar dye produced in the largest quantity was Food Yellow No. 4, accounting for 44.1% during this period.

Chemical Industry↗

PubMed1998

Molecular cloning and functional expression of a fifth-type alpha 2,3-sialyltransferase (mST3Gal V: GM3 synthase).

The cDNA encoding a new type of alpha 2,3-sialyltransferase (mST3Gal V) was cloned from mouse brain cDNA library by PCR-based cloning approach using a pair of degenerate primers deduced from the nucleotide sequence information of mouse ST3Gal III and IV. The predicted amino acid sequence of mST3Gal V showed 27.3% and 26.4% identity to mST3Gal III and IV, respectively. The recombinant soluble mST3Gal V fused with protein-A, which expressed in the culture media of COS-7 cells, showed activity toward lactosylceramide (LacCer), and synthesized GM3. The apparent Km value for LacCer was 9.3 microM. mST3Gal V did not exhibit any activity toward other substrates we tested in this study, including glycolipids, glycoproteins and disaccharides. The mST3Gal V cDNA transfected F28-7 cells, which express large amount of lactosylceramide and very small amount of GM3 at native stage, expressed a large amount of GM3. The ST3Gal V gene was strongly expressed in mouse brain and liver, which contained a large amount of ganglioside. The gene expression seemed to be coincident with ganglioside expression in mouse. Thus, we conclude that mST3Gal V is the fifth-type alpha 2,3-sialyltransferase carrying GM3 synthetic activity.

Amino Acid Sequence↗

PubMed1998

BASH, a novel signaling molecule preferentially expressed in B cells of the bursa of Fabricius.

The bursa of Fabricius is a gut-associated lymphoid organ that is essential for the generation of a diversified B cell repertoire in the chicken. We describe here a novel gene preferentially expressed in bursal B cells. The gene encodes an 85-kDa protein, designated BASH (B cell adaptor containing SH2 domain), that contains N-terminal acidic domains with SH2 domain-binding phosphotyrosine-based motifs, a proline-rich domain, and a C-terminal SH2 domain. BASH shows a substantial sequence similarity to SLP-76, an adaptor protein functioning in TCR-signal transduction. BASH becomes tyrosine-phosphorylated with the B cell Ag receptor (BCR) cross-link or by coexpression with Syk and Lyn and associates with signaling molecules including Syk and a putative chicken Shc homologue. Overexpression of BASH results in suppression of the NF-AT activation induced by BCR-cross-linking. These findings suggest that BASH is involved in BCR-mediated signal transduction and could play a critical role in B cell development in the bursa.

Adaptor Proteins, Signal Transducing↗