Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Surface hydrophobicity, stability, solubility, and kinetics of polymerization were studied using hemoglobins with four different amino acids at the beta 6 position: Hb A (Glu beta 6), Hb C (Lys beta 6), Hb Machida (Gln beta 6), and Hb S (Val beta 6). The surface hydrophobicity increased in the order of Hb C, Hb A, Hb Machida, and Hb S, coinciding with the hydrophobicity of the amino acid at the beta 6 position. Solubility of the oxy-form of these hemoglobins decreased in relation to increases in their surface hydrophobicity, suggesting that the solubility is controlled by the strength of hydrophobicity of the amino acid at the beta 6 position. The solubility of the oxy-form of these hemoglobins is always higher than that of the deoxy-form. There is a similar linear relationship between the solubility and surface hydrophobicity among deoxyhemoglobins A, C, and Machida. However, the solubility of deoxy-Hb S deviated significantly from the expected value, indicating that the extremely low solubility of deoxy-Hb S is not directly related to the hydrophobicity of the beta 6 valine. Kinetic studies on the polymerization of deoxy-Hb Machida revealed a distinct delay time prior to polymerization. This confirms our previous hypothesis that beta 6 valine is not responsible for the delay time prior to gelation. The kinetics of the polymerization of 1:1 mixtures of sickle and non-sickle hemoglobins were similar to those of pure Hb S, suggesting that only one of the two beta 6 valines is involved in an intermolecular contact. In mixtures of equal amounts of Hb S and Hb A, Hb C, or Hb Machida, half of the asymmetrical AS, SC, and S-Machida hybrid hemoglobins behaved like Hb S during nucleation, while the other half behaved like the non-sickle hemoglobin.
J Biol Chem 1987 Sep 25
PMID:Effect of amino acid at the beta 6 position on surface hydrophobicity, stability, solubility, and the kinetics of polymerization of hemoglobin. Comparisons among Hb A (Glu beta 6), Hb C (Lys beta 6), Hb Machida (Gln beta 6), and Hb S (Val beta 6). 288 54

Combined technique with gated planner thallium (Tl)-201 images and gated cardiac pool images were applied to evaluate cardiac hypertrophy and regional wall motion for 13 persons. These subjects consisted of four normal persons, four patients with hypertrophic obstructive cardiomyopathy (HOCM) and five patients with asymmetrical apical hypertrophy (AAH). The Tl-201 image and cardiac pool image were obtained using the gated technique, in which the R-R interval was divided into 16 parts, in the 30 degree left anterior oblique projection. The region of interest (ROI) in the left ventricle was defined by the threshold method and radially divided into 16 segments in the center of the left ventricular area. The count ratio, considered as an indicator of regional myocardial contractility, was calculated using the formula (EDC-ESC/EDC) in each ROI. The functional image and histogram, an indicators of the synchronization of left ventricular wall motion, were constructed by the fluctuation of the phase distribution in volume curves of Tl-201. These indicators were evaluated for the normals and the two groups of patients. The count ratio indicated a higher value in the region of the left ventricular free wall and the cardiac apex in the normals, HOCM and AAH. The count ratio at the septal region indicated a lower value in HOCM, in spite of marked septal hypertrophy. A significant fluctuation of the phase distribution was demonstrated in the septal region in HOCM, and also at the apical region in AAH.(ABSTRACT TRUNCATED AT 250 WORDS)
J Cardiogr 1985 Sep
PMID:[Assessment of regional wall motion of the hypertrophic heart by phase analysis of thallium-201 images]. 294 7

The combination of the systolic anterior motion of the anterior leaflet of the mitral valve and/or the mitral annular calcification in the case of asymmetrical septal hypertrophy has been fully recognized. However, in concentric left ventricular hypertrophy the systolic anterior motion of the anterior mitral valve and the massive posterior submitral calcification have not been commonly reported. We present a case with mild concentric left ventricular hypertrophy and massive posterior submitral calcification which displace the entire mitral ring anteriorly, namely, toward the left ventricular outflow tract. In this case, typical left ventricular outflow obstruction with systolic anterior motion of the anterior mitral valve was seen. Thus, we considered that this rare condition may have contributed to the formation of the systolic anterior motion of the anterior mitral leaflet in this case. We have provided additional information regarding the possible causes of systolic outflow obstruction in hypertrophic obstructive cardiomyopathy.
J UOEH 1986 Sep 01
PMID:Left ventricular outflow tract obstruction due to mitral annulus calcification in patient with mild concentric left ventricular hypertrophy. 294 43

Nonpigmenting fixed drug eruption is a distinctive, clinically recognizable entity. Characteristically, the lesions are large, symmetrical, well-circumscribed tender erythematous plaques that suddenly appear and reappear in exactly the same sites. They fade without pigmentation or any other trace over a 2- to 3-week period. Management requires recognition and avoidance of the responsible drug. Acute attacks may call for short-term systemic steroid therapy. Three examples of this overlooked reaction pattern are presented. The first case was associated with Night-Time cold formula and was proved by specific component challenge to be due to d-pseudoephedrine hydrochloride. The second case was due to PediaCare 3 Children's Cough Syrup, also containing d-pseudoephedrine hydrochloride. The third case was due to Visine eye drops, which contain an imidazole derivative, tetrahydrozoline. There is a need to increase our awareness that fixed drug eruptions come in two very different clinical forms: the classic pigmenting asymmetrical form, and the nonpigmenting symmetrical erythematous plaque form, in both of which the patient will give a history of the eruption being recurrent in the same area.
J Am Acad Dermatol 1987 Sep
PMID:Nonpigmenting fixed drug eruption as a distinctive reaction pattern: examples caused by sensitivity to pseudoephedrine hydrochloride and tetrahydrozoline. 1221 20

We experienced three cases of unusual cardiac hypertrophy of the right ventricle or interventricular septum with severe congestive heart failure in the neonatal period. One patient had congenital heart disease consisting of membranous tricuspid atresia, absent pulmonary valve, patent ductus arteriosus, left single coronary artery and a hypoplastic pouch-like right ventricle. Very marked cardiac hypertrophy was observed in the right ventricle and interventricular septum. Histologically, there was no appreciable disorganization of the cardiac muscle. The etiology of the unusual hypertrophy of cardiac muscle in this patient is uncertain. The other two patients had asymmetrical septal hypertrophy of the left ventricle evidenced by two-dimensional echocardiography. Cardiac catheterization was performed for these two patients. There was no evidence of congenital heart disease; however, one patient had a significant pressure gradient in the outflow tracts of the left and right ventricles. These two patients' faces appeared unusual and they had minor anomalies of their fingers and ears. This unusual cardiac hypertrophy associated with cardiac anomalies and minor anomalies of the face and extremities comprise a specific type of cardiomyopathy in neonates. This should be distinguished from hypertropic cardiomyopathy of older children and adults.
J Cardiogr 1986 Sep
PMID:[Unusual cardiac hypertrophy in neonates with congestive heart failure: report of three cases]. 295 60

Four patients with Fabry's disease diagnosed by right ventricular endomyocardial biopsy had cardiac manifestations simulating hypertrophic cardiomyopathy (HCM). Case 1: A 51-year-old woman, whose elder sister had congestive heart failure, was hospitalized for exertional dyspnea and cardiomegaly. Her electrocardiogram (ECG) showed a short PQ interval (0.10 sec) and left ventricular hypertrophy. Her echocardiogram (Echo) showed moderate symmetrical hypertrophy of the left ventricle (IVST/PWT = 18 mm/17 mm). Case 2: A 32-year-old woman, whose elder sister had an abnormal ECG, was hospitalized for the ECG abnormalities consisting of a short PQ interval (0.10 sec) and ST-T changes in the left precordial leads. The Echo revealed mild symmetrical hypertrophy of the left ventricle (IVST = 13 mm, PWT = 13 mm). Case 3: A 44-year-old man was hospitalized for his ECG suggestive of left ventricular hypertrophy, and his Echo showed asymmetrical septal hypertrophy (ASH; IVST = 22 mm). Case 4: A 51-year-old man was hospitalized for his ECG showing high voltage in the left precordial leads, and his Echo showed ASH (IVST = 20 mm). The cardiac histopathological findings of these cases included cytoplasmic vacuolization by light microscopy, and electron-dense deposits consisting of parallel or concentric lamellae with periodic spacing, suggesting Fabry's disease. The urinary glycolipids of Case 1 were increased biochemically; then the diagnosis of Fabry's disease was confirmed. Cardiac hypertrophy in Fabry's disease has many aspects, because the histopathological changes and clinical manifestations are determined by genetic factors. It was concluded that Fabry's disease may be concealed in some patients with the clinical diagnosis of HCM.
J Cardiol 1988 Sep
PMID:[Four cases of Fabry's disease mimicking hypertrophic cardiomyopathy]. 297 98

In a series of 50 cases in which nerve and/or muscle microvasculitis was seen on biopsy, seven were associated with malignancy. In two cases, the cancer was found after the discovery of microvasculitis. All patients exhibited sensory-motor neuropathy, which was often painful and asymmetrical, with a progressive course. ESR and CSF protein levels were always elevated. Motor conduction velocity was slightly reduced in three cases, unmeasurable in one case, and normal in three. Cancers involved were adenocarcinoma in five cases (three prostate and two lung), Hodgkin's disease in one and immunoblastic lymphadenopathy in one. A thorough search for cancer should be performed when microvasculitis is seen in nerve or muscle biopsy specimens, especially when ESR and CSF protein levels are elevated.
J Neurol Neurosurg Psychiatry 1986 Sep
PMID:Nerve and muscle microvasculitis in peripheral neuropathy: a remote effect of cancer? 302 Jan 78

The amino terminal sequence of the 34 kD nucleolar protein B-36 isolated from the slime mold Physarum polycephalum has been determined. This portion of B-36 is rich in glycine, phenylalanine and the modified amino acid asymmetrical dimethylarginine (DMA) and is 65% identical to that for fibrillarin, a similar and potentially homologous 34 kD nucleolar protein from rat. The terminus of B-36 contains an interesting nine amino acid sequence, Gly-DMA-Gly-Gly-Phe-Gly-Gly-DMA-Gly, which is precisely repeated three times in the 110 kD nucleolar protein nucleolin. Similar sequences have also been reported in a yeast nucleolar protein (SSB-1) and several hnRNP proteins (rat A1 and brine shrimp GRP33). The conserved nature of this unusual sequence is suggestive of an important function which may include RNA-binding since several of these proteins share this feature.
Biochem Biophys Res Commun 1988 Sep 30
PMID:The nucleolar protein, B-36, contains a glycine and dimethylarginine-rich sequence conserved in several other nuclear RNA-binding proteins. 314 Aug 6

The enzymatic transfer of the sugar portion from UDP-N-acetylgalactosamine to pyridylamino (PA) lacto-N-fucopentaose I (Fuc alpha 1-2Gal beta 1-3GlcNAc beta 1-4Glc-PA) was detected by high-performance liquid chromatography. Separation of the fluorescent product from the fluorescence-labeled acceptor was achieved within 10 min by reversed-phase high-performance liquid chromatography. Rat stomach enzyme activity was detected in the microsomal fraction from antrum but not corpus. Ohara et al. (1986, Comp. Biochem. Physiol. 83B, 273-275) reported that the N-acetylgalactosamine content in antrum mucin was greater than that in corpus mucin and antrum mucin had strong blood group A activity. The prominent asymmetrical distribution of the enzyme detected here well supports these findings. The elution position of the fluorescent product was the same as that of the product formed by the action of type A human serum toward the acceptor. Its hydrolysis by alpha-N-acetylgalactosaminidase yielded the acceptor. It is thus evident that the detected enzyme is the same as that producing the blood group A structure.
Anal Biochem 1988 Sep
PMID:Detection of UDP-N-acetylgalactosamine-oligosaccharide N-acetylgalactosaminyltransferase activity in rat stomach and human serum by high-performance liquid chromatography. 314 99

In this study, tibial ray deficiency was used as a model of longitudinal ray deficiency and the pathogenesis of the oligodactyly of longitudinal ray deficiency was investigated. Tibial ray deficiency was induced in rat fetuses by a single oral administration of myleran to pregnant rats on day 10 of pregnancy. The dose of myleran administered was 40 mg/kg of body weight. In order to observe the process of formation of the foot plates and digital rays of tibial ray deficiency, histological examination was done. The period of development studied was from day 12 to 16 of pregnancy. A series of fetuses was surgically removed from their mothers at 12 hours' interval. Histological examinations of the hind limbbuds and foot plates were performed quantitatively. The formation of hind limbbuds was detected on day 12 in myleran treated group as in control group. The formation of digital rays in the myleran treated group was initially observed on day 14.5, 12 hours later than control. The size of hind limbbuds and the density of mesenchymal cells in hind limbbuds treated by myleran was smaller than control and the hypoplasia of the limbbuds was maximum on day 14. The shape of foot plates treated by myleran was asymmetrical compared with the symmetrical limbbuds in controls. Concerning tissue damage in limbbuds, dead cells of ectoderm were distributed on the top of the limbbuds on day 12 and they disappeared 24 hours later. On the other hand, dead cells of mesenchyme were scattered in hind limbbuds on day 12.(ABSTRACT TRUNCATED AT 250 WORDS)
Handchir Mikrochir Plast Chir 1988 Sep
PMID:Histological analysis of myleran induced oligodactyly of longitudinal deficiency in rats. 318 26


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