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S. Pagliarani - One of the best experts on this subject based on the ideXlab platform.
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glucose free high Protein Diet improves hepatomegaly and exercise intolerance in glycogen storage disease type iii mice
Biochimica et Biophysica Acta, 2018Co-Authors: S. Pagliarani, S. Lucchiari, G. Ulzi, M. Ripolone, R. Violano, F. Fortunato, A. Bordoni, S. Corti, M. MoggioAbstract:Abstract Glycogen disease type III (GSDIII), a rare incurable autosomal recessive disorder due to glycogen debranching enzyme deficiency, presents with liver, heart and skeletal muscle impairment, hepatomegaly and ketotic hypoglycemia. Muscle weakness usually worsens to fixed myopathy and cardiac involvement may present in about half of the patients during disease. Management relies on careful follow-up of symptoms and Diet. No common agreement was reached on sugar restriction and treatment in adulthood. We administered two Dietary regimens differing in their Protein and carbohydrate content, high-Protein (HPD) and high-Protein/glucose-free (GFD), to our mouse model of GSDIII, starting at one month of age. Mice were monitored, either by histological, biochemical and molecular analysis and motor functional tests, until 10 months of age. GFD ameliorated muscle performance up to 10 months of age, while HPD showed little improvement only in young mice. In GFD mice, a decreased muscle glycogen content and fiber vacuolization was observed, even in aged animals indicating a protective role of Proteins against skeletal muscle degeneration, at least in some districts. Hepatomegaly was reduced by about 20%. Moreover, the long-term administration of GFD did not worsen serum parameters even after eight months of high-Protein Diet. A decreased phosphofructokinase and pyruvate kinase activities and an increased expression of Krebs cycle and gluconeogenesis genes were seen in the liver of GFD fed mice. Our data show that the concurrent use of Proteins and a strictly controlled glucose supply could reduce muscle wasting, and indicate a better metabolic control in mice with a glucose-free/high-Protein Diet.
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Glucose-free/high-Protein Diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice
'Elsevier BV', 2018Co-Authors: S. Pagliarani, S. Lucchiari, G. Ulzi, M. Ripolone, R. Violano, F. Fortunato, A. Bordoni, S. Corti, M. Moggio, N. BresolinAbstract:Glycogen disease type III (GSDIII), a rare incurable autosomal recessive disorder due to glycogen debranching enzyme deficiency, presents with liver, heart and skeletal muscle impairment, hepatomegaly and ketotic hypoglycemia. Muscle weakness usually worsens to fixed myopathy and cardiac involvement may present in about half of the patients during disease. Management relies on careful follow-up of symptoms and Diet. No common agreement was reached on sugar restriction and treatment in adulthood. We administered two Dietary regimens differing in their Protein and carbohydrate content, high-Protein (HPD) and high-Protein/glucose-free (GFD), to our mouse model of GSDIII, starting at one month of age. Mice were monitored, either by histological, biochemical and molecular analysis and motor functional tests, until 10 months of age. GFD ameliorated muscle performance up to 10 months of age, while HPD showed little improvement only in young mice. In GFD mice, a decreased muscle glycogen content and fiber vacuolization was observed, even in aged animals indicating a protective role of Proteins against skeletal muscle degeneration, at least in some districts. Hepatomegaly was reduced by about 20%. Moreover, the long-term administration of GFD did not worsen serum parameters even after eight months of high-Protein Diet. A decreased phosphofructokinase and pyruvate kinase activities and an increased expression of Krebs cycle and gluconeogenesis genes were seen in the liver of GFD fed mice. Our data show that the concurrent use of Proteins and a strictly controlled glucose supply could reduce muscle wasting, and indicate a better metabolic control in mice with a glucose-free/high-Protein Diet
M. Moggio - One of the best experts on this subject based on the ideXlab platform.
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glucose free high Protein Diet improves hepatomegaly and exercise intolerance in glycogen storage disease type iii mice
Biochimica et Biophysica Acta, 2018Co-Authors: S. Pagliarani, S. Lucchiari, G. Ulzi, M. Ripolone, R. Violano, F. Fortunato, A. Bordoni, S. Corti, M. MoggioAbstract:Abstract Glycogen disease type III (GSDIII), a rare incurable autosomal recessive disorder due to glycogen debranching enzyme deficiency, presents with liver, heart and skeletal muscle impairment, hepatomegaly and ketotic hypoglycemia. Muscle weakness usually worsens to fixed myopathy and cardiac involvement may present in about half of the patients during disease. Management relies on careful follow-up of symptoms and Diet. No common agreement was reached on sugar restriction and treatment in adulthood. We administered two Dietary regimens differing in their Protein and carbohydrate content, high-Protein (HPD) and high-Protein/glucose-free (GFD), to our mouse model of GSDIII, starting at one month of age. Mice were monitored, either by histological, biochemical and molecular analysis and motor functional tests, until 10 months of age. GFD ameliorated muscle performance up to 10 months of age, while HPD showed little improvement only in young mice. In GFD mice, a decreased muscle glycogen content and fiber vacuolization was observed, even in aged animals indicating a protective role of Proteins against skeletal muscle degeneration, at least in some districts. Hepatomegaly was reduced by about 20%. Moreover, the long-term administration of GFD did not worsen serum parameters even after eight months of high-Protein Diet. A decreased phosphofructokinase and pyruvate kinase activities and an increased expression of Krebs cycle and gluconeogenesis genes were seen in the liver of GFD fed mice. Our data show that the concurrent use of Proteins and a strictly controlled glucose supply could reduce muscle wasting, and indicate a better metabolic control in mice with a glucose-free/high-Protein Diet.
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Glucose-free/high-Protein Diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice
'Elsevier BV', 2018Co-Authors: S. Pagliarani, S. Lucchiari, G. Ulzi, M. Ripolone, R. Violano, F. Fortunato, A. Bordoni, S. Corti, M. Moggio, N. BresolinAbstract:Glycogen disease type III (GSDIII), a rare incurable autosomal recessive disorder due to glycogen debranching enzyme deficiency, presents with liver, heart and skeletal muscle impairment, hepatomegaly and ketotic hypoglycemia. Muscle weakness usually worsens to fixed myopathy and cardiac involvement may present in about half of the patients during disease. Management relies on careful follow-up of symptoms and Diet. No common agreement was reached on sugar restriction and treatment in adulthood. We administered two Dietary regimens differing in their Protein and carbohydrate content, high-Protein (HPD) and high-Protein/glucose-free (GFD), to our mouse model of GSDIII, starting at one month of age. Mice were monitored, either by histological, biochemical and molecular analysis and motor functional tests, until 10 months of age. GFD ameliorated muscle performance up to 10 months of age, while HPD showed little improvement only in young mice. In GFD mice, a decreased muscle glycogen content and fiber vacuolization was observed, even in aged animals indicating a protective role of Proteins against skeletal muscle degeneration, at least in some districts. Hepatomegaly was reduced by about 20%. Moreover, the long-term administration of GFD did not worsen serum parameters even after eight months of high-Protein Diet. A decreased phosphofructokinase and pyruvate kinase activities and an increased expression of Krebs cycle and gluconeogenesis genes were seen in the liver of GFD fed mice. Our data show that the concurrent use of Proteins and a strictly controlled glucose supply could reduce muscle wasting, and indicate a better metabolic control in mice with a glucose-free/high-Protein Diet
Chris Ottolenghi - One of the best experts on this subject based on the ideXlab platform.
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successful treatment of severe cardiomyopathy in glycogen storage disease type iii with d l 3 hydroxybutyrate ketogenic and high Protein Diet
Pediatric Research, 2011Co-Authors: Vassili Valayannopoulos, Fanny Bajolle, Jeanbaptiste Arnoux, Sandrine Dubois, Nathalie Sannier, Christiane Baussan, Francois Petit, Philippe Labrune, D Rabier, Chris OttolenghiAbstract:Glycogen storage disease type III (GSD III) due to debranching enzyme deficiency presenting usually with hepatomegaly and hypoglycemia may be responsible for severe cardiomyopathy which is often fatal. Current treatment of GSD III is based on frequent high-carbohydrate meals that have no effect on the cardiomyopathy. We describe a 2-mo-old infant presenting with a familial form of GSD III complicated with cardiomyopathy. As conventional treatment was unable to improve his sister's cardiomyopathy who was deceased at age 11 mo, we proposed an experimental treatment combining the use of synthetic ketone bodies (d,l-3-OH butyrate) as an alternative energy source, 2:1 ketogenic Diet to reduce glucose intake and high-Protein Diet to enhance gluconeogenesis. Twenty-four months after the onset of this treatment, echocardiography showed an improvement of cardiomyopathy. Growth and liver size remained normal, and no side effects were observed. Blood glucose levels remained within the normal range and insulin levels decreased. These findings show that synthetic ketone bodies as well as low-carbohydrate, high-lipid, and high-Protein Diet may be a more beneficial therapeutic choice therapeutic choice for GSD III patients with cardiomyopathy. These encouraging data need to be confirmed in more GSD III patients presenting with cardiac or muscular symptoms.
Adam J Watkins - One of the best experts on this subject based on the ideXlab platform.
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paternal low Protein Diet affects adult offspring cardiovascular and metabolic function in mice
American Journal of Physiology-heart and Circulatory Physiology, 2014Co-Authors: Adam J Watkins, K D SinclairAbstract:Although the association between maternal periconceptional Diet and adult offspring health is well characterised, our understanding of the impact of paternal nutrition at the time of conception on offspring phenotype remains poorly defined. Therefore, we determined the effect of a paternal preconception low Protein Diet (LPD on adult offspring cardiovascular and metabolic health in mice. Male C57BL/6 mice were fed either normal Protein Diet (NPD; 18% casein or LPD (9% casein for 7 wk before mating. At birth, a reduced male-to-female ratio (P = 0.03 and increased male offspring weight (P = 0.009 were observed in litters from LPD compared with NPD stud males with no differences in mean litter size. LPD offspring were heavier than NPD offspring at 2 and 3 wk of age (P <0.02. However, no subsequent differences in body weight were observed. Adult male offspring derived from LPD studs developed relative hypotension (decreased by 9.2 mmHg and elevated heart rate (P <0.05, whereas both male and female offspring displayed vascular dysfunction and impaired glucose tolerance relative to NPD offspring. At cull (24 wk, LPD males had elevated adiposity (P = 0.04, reduced heart-to-body weight ratio (P = 0.04, and elevated circulating TNF-α levels (P = 0.015 compared with NPD males. Transcript expression in offspring heart and liver tissue was reduced for genes involved in calcium signaling (Adcy, Plcb, Prkcb and metabolism (Fto in LPD offspring (P <0.03. These novel data reveal the impact of suboptimal paternal nutrition on adult offspring cardiovascular and metabolic homeostasis, and provide some insight into the underlying regulatory mechanisms.
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low Protein Diet fed exclusively during mouse oocyte maturation leads to behavioural and cardiovascular abnormalities in offspring
The Journal of Physiology, 2008Co-Authors: Adam J Watkins, Adrian Wilkins, Colm Cunningham, Hugh V Perry, Meei J Seet, Clive Osmond, Judith J Eckert, Christopher Torrens, Felino R CagampangAbstract:Early embryonic development is known to be susceptible to maternal undernutrition, leading to a disease-related postnatal phenotype. To determine whether this sensitivity extended into oocyte development, we examined the effect of maternal normal Protein Diet (18% casein; NPD) or isocaloric low Protein Diet (9% casein; LPD) restricted to one ovulatory cycle (3.5 days) prior to natural mating in female MF-1 mice. After mating, all females received NPD for the remainder of gestation and all offspring were litter size adjusted and fed standard chow. No difference in gestation length, litter size, sex ratio or postnatal growth was observed between treatments. Maternal LPD did, however, induce abnormal anxiety-related behaviour in open field activities in male and female offspring (P < 0.05). Maternal LPD offspring also exhibited elevated systolic blood pressure (SBP) in males at 9 and 15 weeks and in both sexes at 21 weeks (P < 0.05). Male LPD offspring hypertension was accompanied by attenuated arterial responsiveness in vitro to vasodilators acetylcholine and isoprenaline (P < 0.05). LPD female offspring adult kidneys were also smaller, but had increased nephron numbers (P < 0.05). Moreover, the relationship between SBP and kidney or heart size or nephron number was altered by Diet treatment (P < 0.05). These data demonstrate the sensitivity of mouse maturing oocytes in vivo to maternal Protein undernutrition and identify both behavioural and cardiovascular postnatal outcomes, indicative of adult disease. These outcomes probably derive from a direct effect of Protein restriction, although indirect stress mechanisms may also be contributory. Similar and distinct postnatal outcomes were observed here compared with maternal LPD treatment during post-fertilization preimplantation development which may reflect the relative contribution of the paternal genome.
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Maternal periconceptional and gestational low Protein Diet affects mouse offspring growth, cardiovascular and adipose phenotype at 1 year of age.
Public Library of Science (PLoS), 2024Co-Authors: Adam J Watkins, Emma S Lucas, Adrian Wilkins, Felino R A Cagampang, Tom P FlemingAbstract:Human and animal studies have revealed a strong association between periconceptional environmental factors, such as poor maternal Diet, and an increased propensity for cardiovascular and metabolic disease in adult offspring. Previously, we reported cardiovascular and physiological effects of maternal low Protein Diet (LPD) fed during discrete periods of periconceptional development on 6-month-old mouse offspring. Here, we extend the analysis in 1 year aging offspring, evaluating mechanisms regulating growth and adiposity. Isocaloric LPD (9% casein) or normal Protein Diet (18% casein; NPD) was fed to female MF-1 mice either exclusively during oocyte maturation (for 3.5 days prior to mating; Egg-LPD, Egg-NPD, respectively), throughout gestation (LPD, NPD) or exclusively during preimplantation development (for 3.5 days post mating; Emb-LPD). LPD and Emb-LPD female offspring were significantly lighter and heavier than NPD females respectively for up to 52 weeks. Egg-LPD, LPD and Emb-LPD offspring displayed significantly elevated systolic blood pressure at 52 weeks compared to respective controls (Egg-NPD, NPD). LPD females had significantly reduced inguinal and retroperitoneal fat pad: body weight ratios compared to NPD females. Expression of the insulin receptor (Insr) and insulin-like growth factor I receptor (Igf1r) in retroperitoneal fat was significantly elevated in Emb-LPD females (P
Claude Remacle - One of the best experts on this subject based on the ideXlab platform.
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effect of maternal low Protein Diet and taurine on the vulnerability of adult wistar rat islets to cytokines
Diabetologia, 2004Co-Authors: S Merezak, Brigitte Reusens, Marietherese Ahn, A Renard, Kevin Goosse, L Kalbe, J Tamaritrodriguez, Claude RemacleAbstract:Aims/hypothesis A maternal low-Protein Diet has been shown to induce an increased susceptibility of fetal islets to cytokines, but this effect can be avoided by maternal taurine supplementation. Here, we question whether these effects persist until adulthood in the offspring, despite the animal having a normal Diet after weaning.
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taurine supplementation to a low Protein Diet during foetal and early postnatal life restores a normal proliferation and apoptosis of rat pancreatic islets
Diabetologia, 2002Co-Authors: S Boujendar, Brigitte Reusens, S Merezak, Marietherese Ahn, Edith Arany, D J Hill, Claude RemacleAbstract:Aims/hypothesis. In our previous studies a low Protein Diet (8% vs 20%) given during foetal and early postnatal life induced abnormal development of the endocrine pancreas; beta-cell mass and islet-cell proliferation were reduced while apoptosis was increased. Taurine, an important amino acid for development was also reduced in maternal and foetal plasma of Protein deficient animals. In this study we aim to evaluate the role of taurine in the alterations observed in rats after a low Protein Diet.
