1,721,036 research outputs found
Glucose-6-phosphate isomerase deficiency
No full textMost of the metabolic needs of erythrocytes are covered by glycolysis, the oxidative pentose phosphate pathway and the glutathione cycle. Hereditary enzyme deficiencies of all these pathways have been identified, among which glucose-6-phosphate isomerase (GPI) deficiency is the second most frequent erythroenzymopathy in glycolysis, being associated with nonspherocytic haemolytic anaemia of variable severity. This autosomal recessive genetic disorder may be associated in some cases with neurological impairment. GPI is a dimeric enzyme that catalyses the reversible interconversion of fructose-6-phosphate and glucose-6-phosphate. Virtually all the mutant gene products reported are characterized by marked instability and normal substrate affinities, but altered catalytic activity and electrophoretic migration rates. At the nucleotide level, 29 mutations have been reported. This chapter reviews (a) the clinical pattern of the condition; (b) biochemical and molecular studies; (c) structure-function relationships; (d) the molecular basis of neurological dysfunctions sometimes associated with GPI deficiency; and (e) the correlation between the severity of the anaemia and the molecular defect
Zygomycosis in a 13 Year Old Girl with T-NHL
No full textWe report the clinical and pathological findings of an unusual invasive fungal infection in a 13-year-old girl with T-NHL. The diagnosis of disseminated Zygomycosis was made four days after onset of clinical symptoms. Risk factors for Zygomycosis were prolonged neutropenia, corticosteroids, and steroid induced diabetes mellitus
Transient and controllable opening of the blood-brain barrier to cytostatic and antibiotic agents by alkylglycerols in rats
No full textThe blood-brain barrier hinders progress in the chemotherapy of brain tumors due to insufficient penetration of anticancer drugs into the brain tissue. Short-chain alkylglycerols affect the physicochemical proper ties of biological membranes. The enhancement of the blood-brain barrier permeability by intra-arterial administration of alkylglycerols was investigated in tumor-free and C6 astroglioma bearing rats. The antineoplastic agents cisplatin and methotrexate and the antibiotics vancomycin and gentamicin were selectively injected into the right internal carotid artery in the absence and presence of various alkylmono-, alkyldi-, and alkyltri-glycerols. In normal rats the intra-arterial administration of the drugs without alkylglycerols resulted in low drug concentrations in brain tissue. In the presence of alkylglycerols (0.01-0.3 M) a reversible (within minutes) and concentration-dependent enrichment of the coinjected agents was found, preferentially in the ipsilateral hemisphere. The extent of drug accumulation in the brain was modified by changes in the chemical structure of the alkylglycerols. The effect increased with the chain length of the alkyl group, decreased with the number of glycerols, and varied from 2- to more than 230-fold compared to controls. In rats with C6 tumors 1-O-pentylglycerol increased the delivery of methotrexate 18-fold in the tumor, 28-fold in the surrounding brain, 18-fold in the contralateral brain, and 19-fold in the cerebellum compared to controls with methotrexate in the absence of pentyl-glycerol. In conclusion, the intra-arterial administration of alkylglycerols represents a novel and well controllable method for enhanced drug delivery to the brain and to brain tumors
Erufosine-induced apoptosis is blocked by peripheral-type benzodiazepine receptor (PBR) ligands in human glioma cells
No full textErucylphosphohomocholine-induced apoptosis in human glioma cells: role of the oligomycin-sensitive F0 part of mitochondrial H+-ATP-synthase
No full textThe 18 kDa Translocator Protein influences adhesion, migration, and proliferation of glioblastoma cells
No full textAlkylglycerol-mediated increase in drug delivery to the normal brain and to brain tumors in rats: Regulation of drug transfer and comparison with hypertonic mannitol and bradykinin
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