A novel route of ATP synthesis

Smolenski, Ryszard T.; Fabianowska‐Majewska, Krystyna; Montero, Celia; Ja, Duley; Fairbanks, L D; Marlewski, M; Ha, Simmonds

doi:10.1016/0006-2952(92)90161-b

Cited by 26 publications

(20 citation statements)

References 10 publications

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“…ATP cannot be resynthesized via the Hx-IMP-AMP pathway because a mature erythroeyte does not have enzymes transforming IMP into AMP (28). The problem needs ftjrther study on the link between erythrocyte and muscle purine metabolism.…”

Section: Discussionsupporting

confidence: 92%

Effect of Training Load Structure on Purine Metabolism in Middle-Distance Runners

Zieliński¹,

Kusy²,

Rychlewski

2011

Medicine &Amp; Science in Sports &Amp; Exercise

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The effect of anaerobic training on purine metabolism is significant despite of a very short total duration of anaerobic loads. Elevated preexercise HGPRT activity in CP suggests adaptation changes consisting in a "permanent readiness" for purine salvage. The detraining in TP leads to reverse adaptation changes. Probably, plasma Hx concentration and erythrocyte HGPRT activity may be considered as a useful measure of training status.

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Section: Discussionsupporting

confidence: 92%

Effect of Training Load Structure on Purine Metabolism in Middle-Distance Runners

Zieliński¹,

Kusy²,

Rychlewski

2011

Medicine &Amp; Science in Sports &Amp; Exercise

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“…Despite the importance of PRS-I enzyme in de novo purine synthesis, purine nucleotides, specifically ATP can be produced by an alternative pathway utilizing S -adenosylmethionine (SAM) as a substrate (Montero et al, 1990; Simmonds et al, 1989; Smolenski et al, 1991, 1992). Methyltransferases are responsible for conversion of SAM into S-adenosylhomocysteine, which is catabolized by hydrolysis to adenosine and L-homocysteine in a reaction catalysed by S-adenosylhomocysteine hydrolase (AHCY) (EC 3.3.1.1) (Kanehisa & Goto, 2000; de Brouwer et al, 2010), or SAM can be converted directly to adenine, through the polyamine pathway (Palella et al, 1980; Smolenski et al, 1992).…”

Section: Management and Treatment Of Patients With Prps1 Defectsmentioning

confidence: 99%

“…Methyltransferases are responsible for conversion of SAM into S-adenosylhomocysteine, which is catabolized by hydrolysis to adenosine and L-homocysteine in a reaction catalysed by S-adenosylhomocysteine hydrolase (AHCY) (EC 3.3.1.1) (Kanehisa & Goto, 2000; de Brouwer et al, 2010), or SAM can be converted directly to adenine, through the polyamine pathway (Palella et al, 1980; Smolenski et al, 1992). Adenine can subsequently be converted to AMP by adenine phosphoribosyltransferase (APRT), utilizing PRPP as a substrate.…”

Section: Management and Treatment Of Patients With Prps1 Defectsmentioning

confidence: 99%

Hearing loss andPRPS1mutations: Wide spectrum of phenotypes and potential therapy

Liu

Xie

Brouwer

et al. 2012

International Journal of Audiology

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Objective The purpose of this review was to evaluate the current literature on phosphoribosylpyrophosphate synthetase 1 (PRPS1)-related diseases and their consequences on hearing function. Design A literature search of peer-reviewed, published journal articles was conducted in online bibliographic databases. Study sample Three databases for medical research were included in this review. Results Mutations in PRPS1 are associated with a spectrum of non-syndromic to syndromic hearing loss. Hearing loss in male patients with PRPS1 mutations is bilateral, moderate to profound, and can be prelingual or postlingual, progressive or non-progressive. Audiogram shapes associated with PRPS1 deafness are usually residual and flat. Female carriers can have unilateral or bilateral hearing impairment. Gain of function mutations in PRPS1 cause a superactivity of the PRS-I protein whereas the loss-of-function mutations result in X-linked nonsyndromic sensorineural deafness type 2 (DFN2), or in syndromic deafness including Arts syndrome and X-linked Charcot-Marie-Tooth disease-5 (CMTX5). Conclusions Lower residual activity in PRS-I leads to a more severe clinical manifestation. Clinical and molecular findings suggest that the four PRPS1 disorders discovered to date belong to the same disease spectrum. Dietary supplementation with S-adenosylmethionine (SAM) appeared to alleviate the symptoms of Arts syndrome patients, suggesting that SAM could compensate for PRS-I deficiency.

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“…1Overview of purine metabolism in human erythrocytes ( straight line ) and out of them ( dotted line ). Based on Simmonds et al 1988 and Smolenski et al 1991, 1992. ADA adenosine deaminase (EC 3.5.4.4), AK adenosine kinase (EC 2.7.1.20), AMPD AMP deaminase (EC 3.5.4.6), APRT adenine phosphoribosyltranferase (EC 2.4.2.7), cN-I cytosolic AMP-specific 5′-nucleotidase (EC 3.2.3.5), cN-II cytosolic IMP and GMP-specific 5′-nucleotidase (EC 3.2.3.5), GK guanosine kinase (EC 2.7.1.73), HGPRT hypoxanthine-guanine phosphorybosyltransferase (EC 2.4.2.8), IK inosine kinase (EC 2.7.1.73), MTAdo methylothioadenosine, 5′-NT-5′-nucleotidase (EC 3.1.3.5), PNP purine nucleoside phosphorylase (EC 2.4.2.1), PRPP 5′-phosphoribosyl 1-pyrophosphate, SAH S-adenosylhomocysteine, SAHH S-adenosylhomocysteine hydrolase (EC 3.3.1.1), SAM S-adenosylmethionine…”

Section: Introductionmentioning

confidence: 99%

Adenine, guanine and pyridine nucleotides in blood during physical exercise and restitution in healthy subjects

et al. 2010

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Maximal physical exertion is accompanied by increased degradation of purine nucleotides in muscles with the products of purine catabolism accumulating in the plasma. Thanks to membrane transporters, these products remain in an equilibrium between the plasma and red blood cells where they may serve as substrates in salvage reactions, contributing to an increase in the concentrations of purine nucleotides. In this study, we measured the concentrations of adenine nucleotides (ATP, ADP, AMP), inosine nucleotides (IMP), guanine nucleotides (GTP, GDP, GMP), and also pyridine nucleotides (NAD, NADP) in red blood cells immediately after standardized physical effort with increasing intensity, and at the 30th min of rest. We also examined the effect of muscular exercise on adenylate (guanylate) energy charge—AEC (GEC), and on the concentration of nucleosides (guanosine, inosine, adenosine) and hypoxanthine. We have shown in this study that a standardized physical exercise with increasing intensity leads to an increase in IMP concentration in red blood cells immediately after the exercise, which with a significant increase in Hyp concentration in the blood suggests that Hyp was included in the IMP pool. Restitution is accompanied by an increase in the ATP/ADP and ADP/AMP ratios, which indicates an increase in the phosphorylation of AMP and ADP to ATP. Physical effort applied in this study did not lead to changes in the concentrations of guanine and pyridine nucleotides in red blood cells.

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A novel route of ATP synthesis

Cited by 26 publications

References 10 publications

Effect of Training Load Structure on Purine Metabolism in Middle-Distance Runners

Effect of Training Load Structure on Purine Metabolism in Middle-Distance Runners

Hearing loss andPRPS1mutations: Wide spectrum of phenotypes and potential therapy

Adenine, guanine and pyridine nucleotides in blood during physical exercise and restitution in healthy subjects

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