PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “PENTOSEPHOSPHATES”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 433 records · Page 24Linked to original sources
PubMed1977

Effectors of glucose-6-phosphate dehydrogenase and 6-phosphogluconate dehydrogenase of rat liver.

The lower Vmax of 6PGDH with respect to G6PDH and its higher sensitivity to inhibition by NADPH, suggest the existence of an imbalance between the two dehydrogenases of the pentose phosphate pathway in rat liver. Possible modulators of these activities, particularly in relation with the inhibition by NADPH in physiological conditions, have been investigated. The results suggest that in both cases the inhibition by NADPH is strictly isosteric and that the relative affinities for the reduced and oxidized forms of the pyridine nucleotide are unaffected by glutathion, the intermediates of the pentose phosphate shunt or some divalent ions.

Animals↗
PubMed1987

HPRT-deficiency associated with normal PRPP concentration and APRT activity.

Deficiencies of HPRT are usually associated with increased concentrations of PRPP and increased levels of APRT activity in erythrocytes. We report the case of a male with a partial deficiency of HPRT in whom these two parameters were normal. The clinical features of this patient were those associated with severe hyperuricaemia and gout. Studies of intact erythrocytes showed rates of incorporation of [14C]hypoxanthine and of [14C]adenine into purine nucleotides which were almost indistinguishable from normal. However, HPRT activity in erythrocyte lysates was only 9% of normal. In cell extracts of cultured lymphoblasts, the HPRT activity was 20% of control values and the APRT activity was normal. The PRPP concentration and the rate of de novo purine synthesis in cultured lymphoblasts were both intermediate between controls and lymphoblasts from patients with the Lesch-Nyhan syndrome.

Adenine Phosphoribosyltransferase↗
PubMed1988

Substrate inhibition of adenosine phosphorylation in adenosine deaminase deficiency and adenosine-mediated inhibition of PP-ribose-P dependent nucleotide synthesis in hypoxanthine phosphoribosyltransferase deficient erythrocytes.

The metabolism of adenosine and its effects on phosphoribosylpyrophosphate, PP-ribose-P, dependent nucleotide synthesis were studied using erythrocytes from patients with adenosine deaminase and hypoxanthine phosphoribosyltransferase deficiency as models. The phosphorylation of adenosine was progressively inhibited by concentrations of adenosine greater than 1 mumol L-1 for control and ADA deficient erythrocytes. There was essentially no initial rate of phosphorylation at 30 mumol L-1 adenosine. Adenosine, 1 mumol L-1, also caused a 60% reduction in PP-ribose-P concentration in ADA deficient erythrocytes. For HPRT deficient erythrocytes in which ADA activity was blocked by coformycin, 10 mumol L-1 inosine stimulated PP-ribose-P dependent nucleotide synthesis from adenine, whereas, 10 mumol L-1 adenosine inhibited nucleotide synthesis. These observations suggest that adenosine phosphorylation and PP-ribose-P dependent nucleotide synthesis are inhibited under conditions in which adenosine accumulates, such as in hereditary or pharmacologically induced ADA deficiency.

Adenosine↗
PubMed1987

Use of intact erythrocytes in the diagnosis of inherited purine and pyrimidine disorders.

This paper reports the detection of five inherited disorders of purine and one of pyrimidine metabolism using intact red blood cells (RBCs) and compares the findings with those from RBC lysate activity. Two different phosphate levels (1 and 18 mmol L-1 Pi) were used to evaluate endogenous PP-ribose-P levels and their generation by PP-ribose-P synthetase. The importance of this dual approach is demonstrated by the following evidence: (a) Six out of eight patients with no detectable hypoxanthine-guanine phosphoribosyltransferase (HGPRT) RBC lysate activity had up to 25% of normal activity in their intact RBCs. Two Lesch-Nyhan patients showed no detectable activity in intact or lysed RBCs. (b) RBC lysates from two heterozygotes for adenosine deaminase (ADA) deficiency also showed no detectable activity, but up to 60% of normal activity using intact RBCs. (c) The existence of an aberrant enzyme in a kindred with a superactive PP-ribose-P synthetase was evident from the fact that intact RBCs failed to respond normally to phosphate activation, despite normal HGPRT and adenine phosphoribosyltransferase (APRT) RBC lysate activity. (d) Raised endogenous PP-ribose-P levels in intact RBCs were demonstrable only in purine nucleoside phosphorylase (PNP) and HGPRT deficiency; levels were normal in APRT deficiency and hereditary oroticaciduria (OPRT/ODC) deficiency. The results indicate that diagnosis from RBC lysate activity alone may be misleading. Intact RBC studies clearly provide a better indication of the functional capacity of the enzyme in vivo. They also show a closer correlation with the clinical phenotype and allow further insight into the associated biochemical abnormalities in some cases.

Adenine Phosphoribosyltransferase↗
PubMed1987

Deficiency of UMP synthase in dairy cattle: a model for hereditary orotic aciduria.

Deficiency of uridine-5'-monophosphate (UMP) synthase in dairy cattle, a condition analogous to human hereditary orotic aciduria, is reviewed with consideration of similarities and differences between the enzyme deficiency in humans and cattle. New findings regarding the bovine condition are reported including presence of the enzyme deficiency in numerous tissues and absence of substantial effects on other aspects of nucleotide metabolism. Specifically, erythrocyte concentration of phosphoribosylpyrophosphate (PRPP) and activities of PRPP synthetase, adenine phosphoribosyltransferase, and hypoxanthine-guanine phosphoribosyltransferase appear to be normal in cattle heterozygous for UMP synthase deficiency.

Adenine Phosphoribosyltransferase↗
PubMed1979

A spectrophotometric method for the determination of 5-phosphoribosyl-1-pyrophosphate.

A new, sensitive, specific and simple spectrophotometric method for the determination of 5-phosphoribosyl-l-pyrophosphate (PRPP) is presented. PRPP is reacted with excess hypoxanthine in the presence of hypoxanthine-guanine phosphoribosyltransferase. At the end of the reaction, PRPP concentration is measured from the extent of conversion of hypoxanthine to inositate. The concentration of the purine base is determined spectrophotometrically in the presence of xanthine oxidase.

Erythrocytes↗
PubMed1985

Capsular variation in experimental strains of Haemophilus influenzae.

This study sought to demonstrate that clinically untypable strains of Haemophilus influenzae are derivable from previously capsulated ones. Penicillin-induced forms were employed to explore in vivo and in vitro a possible mechanism of the reversible cycle vegetative to L-phase revertant. Normal H. influenzae type b (Rab), capsule-deficient strain ATCC 9333, and experimental L-phase and its revertants were used in this investigation. Capsular antigens, polyribose phosphate (PRP) content of each strain was assayed by orcinol and rocket immunoelectrophoretic methods. Intra- and inter-strain PRP differences were statistically analysed. Strain differences between in vivo and in vitro passaged extracts of strain 9333 and 9333 were significant (t-test P less than 0.01). There were also significant differences in vivo and in vitro between penicillin-treated, L-phase infected mouse isolates and penicillin-free, L-phase infected mouse isolates; and also between penicillin-treated, L-phase infected mouse isolates and revertant Rab infected mouse isolates (Mann-Whitney U-test P less than 0.02, P less than 0.01, respectively). These findings suggest that untypable isolates of H. influenzae are derivable from otherwise capsulated strains, depending on decapsulating factors in the microenvironment. Clinical implications of the findings are discussed.

Haemophilus influenzae↗