Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Juvenile rats fed a diet containing 1% lead acetate for 7 weeks, in addition to an impaired growth rate and renal function derangements, suffered malabsorption of glucose and certain amino acids, as assessed by an in vivo perfusion technique. The reduction in glucose absorption ranged between 10% and 31% when the carbohydrate was pumped in concentrations of 2-80 mM. This alteration was compatible with a noncompetitive type of transport inhibition. The intestinal absorption of glycine, lysine, and phenylalanine were, respectively, decreased 22, 18, and 15% when these amino acids were present at 1 mM levels. Sodium transport was severely reduced (57.6 +/- 17.9 (SEM) vs. 124.2 +/- 17.4 muEq/min-cm) and intestinal mucosa (Na+-K+)-ATPase was concomitantly lower in the lead-intoxicated rats (186.4 +/- 19.0 vs 268.4 +/- 29.8 nmol P/min-mg protein). However, this enzyme was not altered in liver and kidney. Furthermore, intestinal mucosa fructose-1,6-diphosphatase, succinic dehydrogenase, pyruvate kinase, and tryptophan hydroxylase were not different in experimental and control animals. These studies substantiate the presence of functional and biochemical abnormalities in the intestinal mucosa of young rats when fed substantial amounts of a soluble lead salt. It is, therefore, reasonable to accept the possibility that physiologic damage occurs in tissues directly subjected to high and persistent levels of a toxic agents, as it occurs in other organs, underscoring the parallelism between transport mechanisms at the renal and intestinal levels.
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PMID:Experimental lead poisoning and intestinal transport of glucose, amino acids, and sodium. 13 38

Jejunal biopsies were obtained from 37 children with cystic fibrosis, 16 with gluten-induced enteropathy, and 18 control subjects for the following studies: (1) disaccharidase activity, (2) L-ALA-L-Phe hydrolase activity, and (3) intestinal uptake of three 14C-labeled amino acids. Values were significantly reduced in the three determinations in patients with gluten-induced enteropathy as compared to control subjects. Lactase and L-ALA-L-Phe hydrolase activities were significantly reduced (p less than 0.01) in CF patients as compared to control subjects. Definite hypolactasia was also observed in 23% of the children with CF. Uptake of lysine was normal in CF patients whereas that of phenylalanine and cycloleucine was reduced as compared to control subjects. This study suggests an intestinal component to the malabsorption of patients with CF.
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PMID:Small bowel mucosal dysfunction in patients with cystic fibrosis. 124 82

Patients with phenylalanine hydroxylase deficiency show increased concentrations of biopterins and neopterins, and reduced concentrations of serotonin and catecholamines, when phenylalanine concentrations are raised. The pterin rise reflects increased synthesis of dihydroneopterin and tetrahydrobiopterin, and the amine fall a reduction in amine synthesis due to inhibition by phenylalanine of tyrosine and tryptophan transport into neurones. The pterin and amine changes appear to be independent of each other and are present in the central nervous system as well as the periphery; they disappear when phenylalanine concentrations are reduced to normal. Patients with arginase deficiency show a similar amine disturbance but have normal pterin levels. The amine changes probably contribute neurological symptoms but pterin disturbance is not known to affect brain function. Patients with defective biopterin metabolism exhibit severely impaired amine synthesis due to tetrahydrobiopterin deficiency. Pterin concentrations vary with the site of the defect. Symptoms include profound hypokinesis and other features of basal ganglia disease. Neither symptoms nor amine changes are relieved by controlling phenylalanine concentrations. Patients with dihydropteridine reductase (DHPR) deficiency accumulate dihydrobiopterins and develop secondary folate deficiency which resembles that occurring in patients with defective 5,10-methylene tetrahydrofolate reductase activity. The latter disorder is also associated with Parkinsonism and defective amine and pterin turnover in the central nervous system, and a demyelinating illness occurs in both disorders. In DHPR deficiency cerebral calcification may develop in a similar distribution to that seen in congenital folate malabsorption and methotrexate toxicity. Symptoms are ameliorated by therapy with 5-formyltetrahydrofolate but exacerbated by folic acid.
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PMID:Pteridines and mono-amines: relevance to neurological damage. 354 Sep 26

A severely affected case of Hartnup disease is reported, where the patient responded rapidly to nicotinamide. This supports the view that all the clinical features, except reduced stature from general nutritional defect, are secondary to tryptophan and nicotinamide deficiency rather than to an unknown toxic factor. Severe malabsorption of both tryptophan and phenylalanine was demonstrated. The dipeptide carnosine was absorbed normally whereas when the two constituent amino acids, beta-alanine and L-histidine, were ingested, absorption of the former was normal but that of the latter was grossly defective. The suggestion is advanced that in cases of Hartnup disease protein nutrition is maintained by intestinal uptake of amino acids as oligopeptides rather than as free amino acids. By contrast, both modes of absorption are probably important in normal subjects. Radiology of the small intestine is abnormal in Hartnup disease when a large amount of protein is admixed with the barium meal.
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PMID:Studies on intestinal absorption of amino acids and a dipeptide in a case of Hartnup disease. 424 31

Reduction of dopamine concentrations in the brains of patients with Parkinsonism, together with reported clinical improvement after the administration of dihydroxyphenylalanine, has led to the hypothesis that impaired hydroxylation of tyrosine may be associated with the disease. To test this hypothesis oral loading tests with L-phenylalanine and tyrosine were carried out in patients and controls. After phenylalanine lower blood levels of this were found in Parkinsonian patients than in controls, but tyrosine levels were the same. After tyrosine lower levels of this were also found in patients compared with controls. It is suggested that these findings indicate a decreased rate of tyrosine utilization in Parkinson's disease together with intestinal malabsorption; the latter is supported by the finding of abnormal D-xylose tolerance in these patients.
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PMID:Oral phenylalanine and tyrosine tolerance tests in Parkinsonian patients. 576 91

In order to elucidate the metabolic disorders which were observed for phenylalanine and tyrosine in protein-energy malnutrition, loads of both these amino acids were given to young mothers who showed all the clinical and biochemical symptoms of malnutrition and to healthy controls of the same age. Loads of phenylalanine resulted in higher blood levels, lower blood tyrosine formed from phenylalanine and higher urinary excretion of secondary phenylalanine and tyrosine metabolites in the patients, the former being due to a phenylalanine hydroxylase deficiency, the latter to concomitant disturbances in tyrosine catabolism. Loads of tyrosine resulted in very high urinary excretion of secondary tyrosine metabolites in two patients, due to a p-hydroxyphenylalanine-oxidase deficiency and to a high urinary excretion of tyrosine but almost normal excretion of secondary metabolites in the third patient, which is very likely to be due to a tyrosine-transaminase deficiency. Blood tyrosine levels remained lower in all patients, which may be explained by tyrosine malabsorption and increased utilization by the depleted tissues. Protein-energy malnutrition leads to enzyme deficiencies similar to those observed in some inborn errors of metabolism.
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PMID:Biochemical study of malnutrition. Part V. Metabolism of phenylalanine and tyrosine. 611 98

Before dietary treatment, no significant differences, except for alanine, were found in the amino acid pattern for 22 young mothers and 33 children with protein-energy malnutrition (PEM), the characteristic feature of the serum pattern being the imbalance between essential and non essential amino acids. Apart from lysine, the essential amino acid levels were all below normal. This imbalance may be detected readily from the abnormal values of some particularly sensitive ratios: phenylalanine/tyrosine, valine/glycine, non essential amino acids/essential amino acids and, above all, serine/threonine. A striking finding was the very low threonine levels in all subjects, including local controls and the extremely low tryptophan levels in malnourished children. Before treatment, almost all the erythrocyte amino acid levels as well as the E/S ratios (erythrocytes/serum) were found to be raised in 9 children, demonstrating their poor clinical status. The urinary amino acid level was similar in both, patients before treatment and local controls. The urinary threonine level was low, like in the serum. A normalisation in most of the amino acid levels in the serum was observed upon dietary rehabilitation although not yet significant in all of them. In urine a similar tendency was observed but it was significant for threonine and methionine only, after 2 weeks treatment. Some additional urinary amino acid assays revealed changes upon two weeks dietary rehabilitation that can be interpreted as an increased production of enzymes affected by PEM as well as a growth of the patients' muscular mass. Increased free amino acid losses in the stools, caused by diarrhoea due to secondary malabsorption, and various viral and bacterial infections accompanying malnutrition, illustrate the severity of the diarrhoea.
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PMID:Biochemical study of malnutrition. Part IV. Determination of amino acids in the serum, erythrocytes, urine and stool ultrafiltrates. 678 95

Six patients with gastrointestinal malabsorption and 12 with alcoholic cirrhosis received total parenteral nutrition for 4 wk. Freamine II, the source of the amino acids, is nearly devoid of cystine and tyrosine. We monitored daily nitrogen balance and other nutritional parameters and 22 plasma amino acids. Malabsorbers had a strongly positive nitrogen balance and improvements in nutritional parameters. Plasma amino acids were maintained within or above their normal fasting ranges. Eight of 12 cirrhotics resembled malabsorbing patients in terms of positive nitrogen balance, improved nutritional parameters, and plasma amino acids. In 4 cirrhotics, nitrogen balance remained negative and nutritional repletion failed to occur. Plasma cystine and tyrosine fell to below 30% of their normal fasting means. In 2 of these patients, oral supplements of cystine and tyrosine were given during the fifth week of parenteral nutrition. Plasma cystine and tyrosine were normalized, nitrogen balance became positive, and other repletion indicators demonstrated recovery. We conclude that in 4 cirrhotics, repletion was blocked by deficiencies of cystine and tyrosine, resulting from hepatic inability to synthesize cystine from methionine and tyrosine from phenylalanine.
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PMID:Hypotyrosinemia, hypocystinemia, and failure to retain nitrogen during total parenteral nutrition of cirrhotic patients. 679 45

The usefulness of optimized and newly elaborated histochemical methods for proteinases is illustrated on two selected substances. DAP IV (Gly-Pro-MNA,FBB,pH 7.2) was discovered in 39% and DAP II (Lys-Ala-MNA,FBB,pH 5.5) in 60% of the lymphocytes of human peripheral blood (ly). The reaction product of such ly differs in quality and quantity. On the ultrastructural level, the reaction product of DAP IV (Gly-Pro-MNA,HNF) was found in cell membranes and lysosomes. Enzyme activity in other areas was probably suppressed during the preparation procedure. Although the number of ly revealed with Lys-Pro-MNA and Phe-Pro-MNA at pH 5.5 and with Lys-Pro-MNA at pH 7.2 is high, these substrates do not distinctly discriminate DAP IV and DAP II. DAP IV occurs exclusively in T lymphocytes. The number of DAP IV-positive ly was not decreased in patients with myelofibrosis, plasmacytoma, chronic granulocytic leukemia, or tricholeukemia. It was, however, greatly reduced in chronic lymphatic leukemia (CLL). In patients with malignant lymphomas other than CLL, ly presence is related to the stage of the disease. Decreased values indicate a more severe stage or a relapse. In the majority of patients with gastric cancer DAP IV-positive ly were decreased. They were normal or increased in patients with peptic ulcer. The assessment of the number of DAP IV-positive ly is a simple method that provides information regarding the condition of patients with malignant lymphomas and gastric carcinoma. Neutrophilic leukocytes and their precursors, and to a lesser extent monocytes, are revealed when N-acetyl-Met-I-naphthyl ester (Ac-Met-N) is used as substrate. Membrane-bound lysosomal and cytosol proteinases were investigated together with disaccharidases in jejunal biopsies of patients with malabsorption syndrome. Activities of all enzymes were affected in patients with celiac disease. According to their impairment enzymes could be arranged: Lactase(L). trehalase (T), brush border endopeptidase (BBEP), gamma-glutamyl transferase (GGT), DAP IV, enzyme(s) cleaving Ac-Mer-N, aminopeptidase A, cytosol peptidases and aminopeptidase M. In the propria, DAP IV is decreased or absent, while GGT and, particularly, DAP II are increased. After a gluten-free diet, activities are restored in a reverse order. BBEP and GGT are useful as auxiliary parameters in the assessment of the damage or differentiation degree of enterocytes. DAP IV is a sensitive indicator of the involvement of the propria.
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PMID:Proteinases in pathology. Usefulness of histochemical methods. 701 84

Blood samples were taken from six overweight women after an overnight fast on three different occasions, before an jejunoileal bypass operation and 1 and 6 months after the operation. The preoperative levels of several plasma free amino acids were significantly elevated, e.g. leucine, isoleucine, valine, lysine, phenylalanine, tyrosine, proline and glutamic acid. One month after the operation all indispensable plasma amino acid concentrations had fallen, in particular the levels of the branched-chain amino acids (BCAA), lysine and tryptophan. Among the dispensable amino acids, plasma tyrosine, arginine and ornithine concentrations were significantly reduced. No further changes of significance were observed in samples taken 5 months later. A close correlation was observed between the plasma levels of retinol-binding protein (RBP) and thyroxine-binding prealbumin (TBPA). One month after the operation the levels of RBP and TBPA had fallen slightly in two subjects and substantially in one subject. A test diet, containing crystalline amino acids, glucose and fat emulsion was given before operation and twice after the operation. Plasma amino acid changes were studied for a period of 2 hours after the meal. The increases in plasma levels following the test meal were lower for many amino acids after the operation. A linear correlation was found between the postprandial increases in BCAA concentrations and the levels of RBP and TBPA. By using complete, carefully defined diets in loading tests, it should be possible to screen for glucose tolerance and amino acid and lipid malabsorption.
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PMID:An oral amino acid loading test before and after intestinal bypass operation for morbid obesity. 713 14


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