Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A diterpenoid-lactone, white thin crystals, C20H24O3, m/z: 312 (M+), mp 222-223 degrees C, UV lambda max (EtOH) 217 (log epsilon 4.36) nm, has been isolated from the ethyl acetate extract of the roots of Tripterygium wilfordii Hook. f., in a yield of 0.025%. Its structure was elucidated by spectral analysis (UV, IR, MS, 1HNMR and 13CNMR) and X-ray SCD. It is the known triptophenolide with revision of structure. Triptophenlolide was shown to have obvious inhibiting effects on lymphocyte and IgG (P less than 0.01) when mice and rats were given ig 1.5 mg/kg. The total complements in blood serum was increased. When BALB/C mice were given ig 1.5 mg/kg, the ear oedema induced by dimethyl benzene was significantly inhibited (P less than 0.01); The ear oedema induced by croton oil in SD rats at a dose of ig 1.0 mg/kg was also significantly inhibited (P less than 0.05). The vitamin C content of the adrenal gland was reduced in mice at a dose of 1.5 mg/kg. The ig LD50 of triptophenolide was greater than 30 mg/kg.
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PMID:[Structure revision of triptophenolide]. 210 74

Sodium dimercaptopropanesulphonate (DMPS) and sodium dimercaptosuccinate (DMS) were discovered to be effective antidotes for acute poisoning of insecticides SCD [sodium ammonium dimethyl-2-(propane-1,3-dithiosulfate) monohydrate], nereistoxin (4-N,N-dimethylamino-1,2-dithiolane) and cartap (dihydronereistoxin dicarbamate). In mice, DMPS (250 mg/kg) or DMS (1000 mg/kg) ip 20 min before SCD increased LD50 of ig SCD from 97 to 374 or 251 mg/kg, respectively. The prophylactic effect of DMPS was better than that of DMS. Administration of DMPS prior to cartap increased LD50 of ig cartap from 130 to 375 mg/kg. The therapeutic effect of DMPS was also demonstrated in SCD-poisoned conscious rabbits. DMPS 62.5 mg/kg or DMS 500 mg/kg iv completely antagonized the neuromuscular blockade and respiratory depression caused by SCD, nereistoxin and cartap in anesthetized rabbits. The antagonism of SCD-induced neuromuscular blockade by cysteine (400 mg/kg, iv) was less effective and of shorter duration than that by DMPS and DMS. Dimercaprol 50 mg/kg im showed little effect on SCD-induced paralysis. The antagonistic actions of sulfhydryl compounds on neuromuscular blockade induced by these insecticides probably belong to chemical antagonism.
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PMID:[Antidotal effects of sulfhydryl compounds on acute poisonings by sodium ammonium dimethyl-2-(propane-1,3-dithiosulfate) monohydrate, nereistoxin and cartap]. 217 10

Patients with hemoglobinopathies can be at risk for significant maternal and fetal morbidity during pregnancy. This is especially true for those gravidas with SCD or certain thalassemia disorders. With intensive management, pregnancy outcome for these women has improved dramatically, and approximates that of the general population. Criteria for diagnosis for many of these conditions are well established. Appropriate therapeutic interventions are more controversial, but, regardless, emphasize the need for a heightened awareness of these disorders and their potential complications.
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PMID:The diagnosis and management of hemoglobinopathies during pregnancy. 218 52

The abnormal susceptibility towards certain infections in SCD patients has a partial explanation in the well described functional defects of the spleen and of the alternative complement pathway; such defects probably account for the etiology of fulminant, often fatal, childhood infections with encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae). On the other hand, the frequent systemic infections with enteric organisms in SCD patients, particularly of the salmonella species, and also with Staphylococcus aureus, are more difficult to explain. We therefore reviewed the potential contribution of neutrophil (PMN) dysfunctions to the increased infective tendency of SCD patients and included some previously unpublished data from our laboratory. While notable discrepancies still exist--and need further clarification--a tentative working hypothesis can be extracted from the available data: dysfunctions of neutrophils affect their locomotion (as reflected by decreased chemotaxis and in vivo migration), their phagocytic processes and their bactericidal performance. The latter concerns the ineffective killing of Staphylococcus aureus, Candida albicans, and Streptococcus pneumoniae. Dysfunctional bactericidal activity, in turn, apparently relates to a poor or at times non-existent PMN oxidative activity, which prevents the prompt disposal of microorganisms. Under certain circumstances salmonella species seem to further paralyze the oxidative machinery of PMNs in SCD. Serum from some patients contains a poorly defined inhibitor, or lacks an enhancing factor, and such serum abnormalities aggravate the existing defects just described. Interestingly recent findings suggest that dysfunctional PMNs may originate from the mandatory demargination of leukocytes secondary to the functional asplenia of SCD; a predominance of non-rosetting (EA-) PMNs among such leukocytes could produce the operational explanation for an exaggerated representation of dysfunctional PMNs in SCD patients with leukocytis.
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PMID:Neutrophil dysfunctions in sickle cell disease. 220 48

The order of phosphatidylcholine (PC) acyl chains in the surface monolayer of very low density lipoproteins (VLDL) and low density lipoproteins (LDL) has been determined from 2H nuclear magnetic resonance order parameters, SCD, using selectively deuterated PC or palmitic acids. From the computer simulated line shapes, we find two distinct phospholipid domains within the amphiphilic monolayer of both VLDL and LDL. In the more ordered domain of LDL, SCD was approximately 0.3 for the "plateau" chain region. The SCD values of VLDL particles are similar to those of LDL for the 5,6- and 11,12-positions, hence we suggest the organization of the more ordered region of VLDL and LDL are similar. The domain of low order in LDL comprises less than 10% of the phospholipid molecules (we do not distinguish between PC and sphingomyelin), having approximately the same order (SCD less than 0.1) as egg PC - sphingomyelin unilamellar vesicles. In VLDL, the domain of low order comprises between approximately 10 and approximately 20% of the phospholipid molecules and the entire acyl chain is in an essentially isotropic environment (SCD less than 0.02). We prepared VLDL-sized microemulsions composed of egg PC, deuterated PC, and triolein to test whether the apoproteins were responsible for creating the two differently organized domains in VLDL and LDL. Surprisingly, these protein-free particles also showed two domains of different order at two temperatures. The high order region, however, is less ordered than in VLDL and LDL. We explain two surface domains of PC in terms of lipid organization and the unique interactions of lipids in the various lipoprotein particles.
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PMID:Dynamic structure of the lower density lipoproteins. II. Deuterium NMR studies of the monolayer of very low and low density lipoproteins. 235 Apr 86

Motor evoked potentials (MEPs) to transcranial stimulation (TCS) and somatosensory evoked potentials to median nerve stimulation (MN-SEPs) were examined in 74 patients affected by multiple sclerosis (MS = 49 cases), amyotrophic lateral sclerosis (ALS = 9 cases), cervical cord lesions (7 cases), Parkinson's disease (PD = 5 cases), Huntington's chorea (HC = 2 cases), Wilson's disease (WD = 1 case), subacute combined degeneration (SCD = 1 case). MN-SEPs were altered in 38% of arms in MS with a higher incidence in clinically affected than in clinically 'silent' arms (= 77.8% vs. 27.5%). MEP alterations were found in 54% of examined arms, mostly because of a prolongation of the motor CCT. This index was invariably altered in the affected arms, whilst it was involved in 40% of the 'silent' ones. Twelve out of 18 arms displayed abnormal MEPs in ALS. These were mainly due to an absent response, even if moderate motor CCT prolongation and 'giant' MEPs were also encountered. MN-SEPs were altered in 3/18 arms. By recording MEPs from proximal and distal upper limb muscles, cues on the level of abnormal propagation were obtained in patients suffering from 'focal' lesions of the spinal cord. Combining SEP records enhanced the diagnostic yield in this field. Both MEPs and SEPs were normal in patients with PD and HC, whilst abnormally prolonged CCTs were found in the case with WD. MEP and SEP recording revealed central propagation abnormalities coupled to a severe clinical picture of the peripheral nerve involvement (as in the case of SCD).
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PMID:Neurophysiological evaluation of the central nervous impulse propagation in patients with sensorimotor disturbances. 245 26

From 1982 to 1986, there were 1230 sudden death cases autopsied in Osaka Medical Examiner's Office. Among them, 810 cases were classified cardiac deaths (SCDs) such as coronary heart disease (77%), cardiomyopathy (7%), valvular disease (3%). All SCD cases were dead within 24 hours after the appearance of the fatal symptoms, and most of them (72%) were supposed to be instantaneous death. Many of the fatal symptoms began in bed (31%), at bath (17%), at toilet (8%) or at work (8%). 34% of them were thought to be healthy by themselves or by their family members before their deaths. Hypertension (38%), coronary heart disease (13%) and diabetes mellitus (11%) were their major past histories recorded. Cardiac lesions such as myocardial hypertrophy, fibrosis and atherosclerosis of coronary artery were frequently admitted in SCD. SCD has been vigorously studied for a long time in various fields including legal medicine, and it has also attracted a great deal of public attention recently. To clarify the causes and the mechanisms of SCD, investigations of the actual situation of SCD cases is essential. Therefore, 1230 autopsy cases of SCD in Osaka Medical Examiner's Office for recent five years were statistically investigated.
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PMID:A statistical study of sudden cardiac death for past five years in Osaka medical, investigated at the Osaka Medical Examiner's Office. 252 Jun 96

Previously we isolated and characterized a differentially expressed gene from mouse 3T3-L1 preadipocytes that encodes stearoyl-CoA desaturase (SCD1; Ntambi, J. M., Buhrow, S. A., Kaestner, K. H., Christy, R. J., Sibley, E., Kelly, T. J., Jr., and Lane, M. D. (1988) J. Biol. Chem. 263, 17291-17300). Genomic Southern blot analysis indicated the existence of another closely related gene. Here we report the isolation and characterization of this gene and the corresponding cDNA which encode a second stearoyl-CoA desaturase, SCD2, 3T3-L1 adipocytes. SCD2 cDNA is 5 kilobase pairs in length and encodes a protein of 358 amino acids with greater than 87% amino acid sequence identity to SCD1. RNase protection analysis reveals a 10-fold increase in the expression of SCD2 mRNA during 3T3-L1 preadipocyte differentiation. SCD2 mRNA is expressed constitutively at a high level in brain, is not expressed in liver, and its expression in kidney, adipose, and lung tissue is increased greatly by shifting mice from a diet containing unsaturated fatty acids to a diet devoid of fat. The tissue distribution and the dietary alteration of SCD1 mRNA expression differs markedly from that of SCD2 mRNA being absent from brain, constitutive in adipose tissue, and subject to negative control in liver by feeding a diet containing unsaturated fatty acids. The SCD2 gene spans approximately 15 kilobase pairs and consists of six exons and five introns, with intron/exon junctions similar to those of SCD1. As determined by primer extension analysis the start site of transcription maps 300 nucleotides upstream of the initiator methionine codon. Unlike the SCD1 gene, SCD2 lacks a typical "TATA" box in the 5'-flanking region, but has two "CCAAT" boxes at positions -90 and -135 relative to the transcription initiation site. The SCD2 promoter contains a 140-base pair sequence (located between nucleotides -54 and -201) which possesses 77% sequence identity to a region (located between nucleotides -472 and -325) in the SCD1 promoter. There is a GC-rich sequence in the SCD2 promoter (at nucleotide -175) similar to the binding site for the nuclear transcription factor Sp1 as well as an element with homology to the core consensus sequence for the glucocorticoid regulatory element position -500 and a potential CCAAT box/enhance binding protein sequence at position -540. The SCD gene family provides a new model system for the study of differentiation-induced as well as tissue-specific metabolite controlled gene expression.
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PMID:Differentiation-induced gene expression in 3T3-L1 preadipocytes. A second differentially expressed gene encoding stearoyl-CoA desaturase. 257 68

In the past few years, routine studies of SCE induction in vivo in fish have been hampered by unreliable SCD techniques. This paper presents a number of modifications of the SCD technique in vivo in Nothobranchius rachowi. Major improvements were obtained by BrdU incorporation from aqueous solutions, short intervals between preparation and staining of slides and post-treatment with HCl. These improvements resulted in a highly reliable SCD procedure in Nothobranchius with a low level base-line SCE frequency (0.90 SCE/metaphase, 0.059 SCE/chromosome). Further research is now directed at gathering additional data on base-line SCE frequencies, establishing the sensitivity of the assay for aqueous solutions of known mutagens, and defining an experimental set-up for optimal statistical evaluation.
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PMID:Some factors affecting optimal differential staining of sister-chromatids in vivo in the fish Nothobranchius rachowi. 258 49

The purpose of this study is to investigate the long-term prognosis of ventricular tachycardia (VT) mainly with respect to sudden death (SCD) in patients with ischemic heart disease (IHD), idiopathic cardiomyopathy (ICM), miscellaneous heart disease (MHD) and idiopathic ventricular tachycardia (IVT). The study included 117 patients with VT (80 male, 37 female). The number of patients with IHD, ICM, MHD and IVT were 40, 18, 26 and 33, respectively. Follow-up was conducted by means of a mailed standardized questionnaire. The mean follow-up period was 46.8 +/- 32.0 months (range from 6 to 125 months). In 24 out of the 117 patients the cause of death was SCD, in 9 there was no sudden cardiac death and in 5 no cardiac death. The other 76 were surviving. The number of SCD in IHD, ICM, MHD and IVT was 14/40 (35%), 4/17 (24%), 6/25 (24%) and zero (0%), respectively. The number of having had syncope in IHD, ICM, MHD and IVT was 19/40 (48%), 7/18 (39%), 6/26 (23%) and 6/33 (18%), respectively. Out of the 19 IHD patients with syncope, 15 had had ventricular fibrillation (VF). As for IVT with syncope, only one of the 6 had VF, which was induced by a disopyramide injection. In IVT, the patients with syncope had a significantly higher VT rate than those without syncope (p less than 0.01). There were no significant differences in the electrocardiographical high risk parameters for SCD, the age, follow-up periods, the presence or absence of VF and ejection fraction between the SCD and the surviving groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long-term prognostic assessment of ventricular tachycardia with respect to sudden death in patients with and without overt heart disease. 263 26


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