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NOX, the main regulator in oxidative stress in experimental models of phenylketonuria?

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Veröffentlicht/Copyright: 22. April 2013
Journal of Pediatric Endocrinology and Metabolism
Aus der Zeitschrift Journal of Pediatric Endocrinology and Metabolism Band 26 Heft 7-8

Abstract

Aim: To reveal the role of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NOX) in the integration of the redox signal in the oxidative molecular regulation mechanism in phenylketonuria (PKU).

Methods: The blood samples were obtained from Pahenu2-BTBR PKU and wild-type mice, respectively. Phe concentration, total antioxidant capacity (T-AOC), glutathione (GSH) and maleic dialdehyde (MDA) were analyzed. After collection of the mononuclear cells, reverse transcription polymerase chain reaction (RT-PCR) for NOX was performed. In addition, NOX activity and superoxide in mononuclear cells were determined.

Results: Compared to the control group, Phe concentration, T-AOC and MDA were markedly increased in PKU mice (p<0.01, p<0.05, p<0.01, respectively). However, the GSH level in PKU mice was less than that in control group (p<0.05). The mRNA level of subunits of NOX included p47phox and p67phox, were increased in PKU mice (p<0.05), however, the gp91phox had no obvious change in the two groups (p>0.05). NOX activity and superoxide were also remarkably elevated in PKU mice (p<0.05).

Conclusion: NOX may play an important role in the integration of the redox signal in the oxidative molecular regulation mechanism in PKU.

Keywords: mononuclear cells; NOX; phenylketonuria; superoxide
Corresponding author: Xuefan Gu, Department of Pediatric Endocrinology and Genetical Metabolism, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai Institute for Pediatric Research, 1665# Kongjiang Road, Shanghai 200092, China, Phone: +86-21-65791316, Fax: +86-21-65791316

This work was supported by grants from the Major Program of Shanghai Committee of Science and Technology (11dz195030), The National Natural Science Foundation of China (81070700) and The National Key Technology R&D Program (2012BAI09B04).

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Received: 2012-12-5
Accepted: 2013-2-18
Published Online: 2013-04-22
Published in Print: 2013-08-01

©2013 by Walter de Gruyter Berlin Boston

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https://doi.org/10.1515/jpem-2012-0387
Schlagwörter für diesen Artikel
mononuclear cells; NOX; phenylketonuria; superoxide

Artikel in diesem Heft

  1. Masthead
  2. Masthead
  3. Editorials
  4. Editorial
  5. Precocious puberty and diagnostic tests
  6. Review article
  7. The influence of carbohydrate quality on cardiovascular disease, the metabolic syndrome, type 2 diabetes, and obesity – an overview
  8. Original Articles
  9. Triptorelin depot stimulation test for central precocious puberty
  10. Umbilical cord and fifth-day serum vaspin concentrations in small-, appropriate-, and large-for-gestational age neonates
  11. Growth patterns in pubertal HIV-infected adolescents and their correlation with cytokines, IGF-1, IGFBP-1, and IGFBP-3
  12. Estrogen receptor α gene analysis in girls with central precocious puberty
  13. Trends of body composition among adolescents according to maturation stage and body mass index
  14. Oxidized low-density lipoprotein levels and carotid intima-media thickness as markers of early atherosclerosis in prepubertal obese children
  15. Interaction of bone mineral density, adipokines and hormones in obese adolescents girls submitted in an interdisciplinary therapy
  16. The contribution of art therapy in poorly controlled youth with type 1 diabetes mellitus
  17. NOX, the main regulator in oxidative stress in experimental models of phenylketonuria?
  18. Changes in growth pattern, leptin ghrelin and neuropeptide Y levels after adenotonsillectomy in prepubertal children
  19. Impact of inborn errors of metabolism on admission in a neonatal intensive care unit: a 4-year report
  20. Correlation between bone mineral density measured by peripheral and central dual energy X-ray absorptiometry in healthy Indian children and adolescents aged 10–18 years
  21. Thyroid-stimulating hormone (TSH) level in nutritionally obese children and metabolic co-morbidity
  22. Hepatocyte growth factor as an indicator of neonatal maturity
  23. Pathophysiology of critical illness hyperglycemia in children
  24. The influence of exposure to maternal diabetes in utero on the rate of decline in β-cell function among youth with diabetes
  25. Clinical and molecular characterization of Chilean patients with Léri-Weill dyschondrosteosis
  26. Abdominal aorta intima media thickness in obese children
  27. Patient reports
  28. KCNJ11 in-frame 15-bp deletion leading to glibenclamide- responsive neonatal diabetes mellitus in a Chinese child
  29. Hoffmann’s syndrome and pituitary hyperplasia in an adolescent secondary to Hashimoto thyroiditis
  30. Late diagnosis of Cushing’s disease in a child: what lessons can be learned?1)
  31. Variable phenotype in five patients with Wolcott-Rallison syndrome due to the same EIF2AK3 (c.1259delA) mutation
  32. Recurrent infantile hypoglycemia due to combined fructose-1,6-diphosphatase deficiency and growth hormone deficiency
  33. A previously undescribed mutation detected by sequence analysis of CYP21A2 gene in an infant with salt wasting congenital adrenal hyperplasia
  34. Transient neonatal hyperparathyroidism: a presenting feature of sialidosis type II
  35. Effect of recombinant insulin-like growth factor-1 treatment on short-term linear growth in a child with Majewski osteodysplastic primordial dwarfism type II and hepatic insufficiency
  36. Duplication of dosage sensitive sex reversal area in a 46, XY patient with normal sex determining region of Y causing complete sex reversal
  37. The search for ectopic ACTH production in a 9-year-old boy
  38. Development of antibodies against growth hormone (GH) during rhGH therapy in a girl with idiopathic GH deficiency: a case report
  39. Ovotesticular disorder of sexual development and a rare 46,XX/47,XXY karyotype
  40. Brachydactyly mental retardation syndrome in differential diagnosis of pseudopseudohypoparathyroidism
  41. Letters to the Editors
  42. How much truth is there in the association between endometriosis and atherosclerosis?
  43. Delayed cord clamping in plethoric term neonates of diabetic mothers: friend or foe?
  44. Meetings
  45. Meetings Calendar
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