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. 1989 Aug;52(8):954–961. doi: 10.1136/jnnp.52.8.954

The effect of a high protein diet on leucine and alanine turnover in acid maltase deficiency.

A M Umpleby 1, P S Trend 1, D Chubb 1, J V Conaglen 1, C D Williams 1, R Hesp 1, I N Scobie 1, C M Wiles 1, G Spencer 1, P H Sönksen 1
PMCID: PMC1031833  PMID: 2507747

Abstract

Leucine and alanine production rate was measured in 5 patients with acid maltase deficiency in the postabsorptive state, following 6 months on a normal diet with placebo and 6 months on an isocaloric high protein diet (16-22% protein). Whole body leucine production rate, a measure of protein degradation, expressed in terms of lean body mass was significantly greater than in five control subjects. Following the high protein diet, leucine production rate was decreased in four of the five patients but this was not statistically significant. Alanine production rate expressed in terms of lean body mass was significantly greater than in control subjects. After the high protein diet, alanine production rate and concentration were significantly decreased (p less than 0.05). There were no significant improvements in any of the clinically relevant variables measured in these patients. It is possible that a larger increase in protein intake over a longer time period may have a clinical effect.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. AGOSTONI E., RAHN H. Abdominal and thoracic pressures at different lung volumes. J Appl Physiol. 1960 Nov;15:1087–1092. doi: 10.1152/jappl.1960.15.6.1087. [DOI] [PubMed] [Google Scholar]
  2. Angelini C., Engel A. G. Comparative study of acid maltase deficiency. Biochemical differences between infantile, childhood, and adult types. Arch Neurol. 1972 Apr;26(4):344–349. doi: 10.1001/archneur.1972.00490100074007. [DOI] [PubMed] [Google Scholar]
  3. Angelini C., Engel A. G., Titus J. L. Adult acid maltase deficiency. Abnormalities in fibroblasts cultured from patients. N Engl J Med. 1972 Nov 9;287(19):948–951. doi: 10.1056/NEJM197211092871902. [DOI] [PubMed] [Google Scholar]
  4. Birkhahn R. H., Long C. L., Fitkin D., Geiger J. W., Blakemore W. S. Effects of major skeletal trauma on whole body protein turnover in man measured by L-[1,14C]-leucine. Surgery. 1980 Aug;88(2):294–300. [PubMed] [Google Scholar]
  5. Björntorp P., Schröder G., Orndahl G. Carbohydrate and lipid metabolism in relation to body composition in myotonic dystrophy. Diabetes. 1973 Apr;22(4):238–242. doi: 10.2337/diab.22.4.238. [DOI] [PubMed] [Google Scholar]
  6. Black L. F., Hyatt R. E. Maximal static respiratory pressures in generalized neuromuscular disease. Am Rev Respir Dis. 1971 May;103(5):641–650. doi: 10.1164/arrd.1971.103.5.641. [DOI] [PubMed] [Google Scholar]
  7. Boddy K., King P. C., Hume R., Weyers E. The relation of total body potassium to height, weight, and age in normal adults. J Clin Pathol. 1972 Jun;25(6):512–517. doi: 10.1136/jcp.25.6.512. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Carruthers M., Young D. A. Free fatty acid estimation by a semi-automated fluorimetric method. Clin Chim Acta. 1973 Dec 27;49(3):341–348. doi: 10.1016/0009-8981(73)90231-3. [DOI] [PubMed] [Google Scholar]
  9. DOLE V. P., MEINERTZ H. Microdetermination of long-chain fatty acids in plasma and tissues. J Biol Chem. 1960 Sep;235:2595–2599. [PubMed] [Google Scholar]
  10. Dreyfus J. C., Poënaru L. Alpha glucosidases in white blood cells, with reference to the detection of acid alpha 1-4 glucosidase deficiency. Biochem Biophys Res Commun. 1978 Nov 29;85(2):615–622. doi: 10.1016/0006-291x(78)91207-x. [DOI] [PubMed] [Google Scholar]
  11. Emery P. W., Edwards R. H., Rennie M. J., Souhami R. L., Halliday D. Protein synthesis in muscle measured in vivo in cachectic patients with cancer. Br Med J (Clin Res Ed) 1984 Sep 8;289(6445):584–586. doi: 10.1136/bmj.289.6445.584. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Engel A. G., Gomez M. R., Seybold M. E., Lambert E. H. The spectrum and diagnosis of acid maltase deficiency. Neurology. 1973 Jan;23(1):95–106. doi: 10.1212/wnl.23.1.95. [DOI] [PubMed] [Google Scholar]
  13. Forlani G., Vannini P., Marchesini G., Zoli M., Ciavarella A., Pisi E. Insulin-dependent metabolism of branched-chain amino acids in obesity. Metabolism. 1984 Feb;33(2):147–150. doi: 10.1016/0026-0495(84)90127-6. [DOI] [PubMed] [Google Scholar]
  14. HOHORST H. J., KREUTZ F. H., BUECHER T. [On the metabolite content and the metabolite concentration in the liver of the rat]. Biochem Z. 1959;332:18–46. [PubMed] [Google Scholar]
  15. Hudgson P., Fulthorpe J. J. The pathology of type II skeletal muscle glycogenosis. A light and electron-microscopic study. J Pathol. 1975 Jul;116(3):139–147. doi: 10.1002/path.1711160303. [DOI] [PubMed] [Google Scholar]
  16. Isaacs H., Savage N., Badenhorst M., Whistler T. Acid maltase deficiency: a case study and review of the pathophysiological changes and proposed therapeutic measures. J Neurol Neurosurg Psychiatry. 1986 Sep;49(9):1011–1018. doi: 10.1136/jnnp.49.9.1011. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Keunen R. W., Lambregts P. C., Op de Coul A. A., Joosten E. M. Respiratory failure as initial symptom of acid maltase deficiency. J Neurol Neurosurg Psychiatry. 1984 May;47(5):549–552. doi: 10.1136/jnnp.47.5.549. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Kien C. L., Young V. R., Rohrbaugh D. K., Burke J. F. Increased rates of whole body protein synthesis and breakdown in children recovering from burns. Ann Surg. 1978 Apr;187(4):383–391. doi: 10.1097/00000658-197804000-00007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Motil K. J., Matthews D. E., Bier D. M., Burke J. F., Munro H. N., Young V. R. Whole-body leucine and lysine metabolism: response to dietary protein intake in young men. Am J Physiol. 1981 Jun;240(6):E712–E721. doi: 10.1152/ajpendo.1981.240.6.E712. [DOI] [PubMed] [Google Scholar]
  20. Moxley R. T., Griggs R. C., Forbes G. B., Goldblatt D., Donohoe K. Influence of muscle wasting on oral glucose tolerance testing. Clin Sci (Lond) 1983 Jun;64(6):601–609. doi: 10.1042/cs0640601. [DOI] [PubMed] [Google Scholar]
  21. Rennie M. J., Edwards R. H., Millward D. J., Wolman S. L., Halliday D., Matthews D. E. Effects of Duchenne muscular dystrophy on muscle protein synthesis. Nature. 1982 Mar 11;296(5853):165–167. doi: 10.1038/296165a0. [DOI] [PubMed] [Google Scholar]
  22. Rennie M. J. Muscle protein turnover and the wasting due to injury and disease. Br Med Bull. 1985 Jul;41(3):257–264. doi: 10.1093/oxfordjournals.bmb.a072060. [DOI] [PubMed] [Google Scholar]
  23. STEINKE J., TYLER H. R. THE ASSOCIATION OF AMYOTROPHIC LATERAL SCLEROSIS (MOTOR NEURON DISEASE) AND CARBOHYDRATE INTOLERANCE, A CLINICAL STUDY. Metabolism. 1964 Nov;13:1376–1381. doi: 10.1016/0026-0495(64)90158-1. [DOI] [PubMed] [Google Scholar]
  24. Slonim A. E., Coleman R. A., McElligot M. A., Najjar J., Hirschhorn K., Labadie G. U., Mrak R., Evans O. B., Shipp E., Presson R. Improvement of muscle function in acid maltase deficiency by high-protein therapy. Neurology. 1983 Jan;33(1):34–38. doi: 10.1212/wnl.33.1.34. [DOI] [PubMed] [Google Scholar]
  25. Smith T., Hesp R., Mackenzie J. Total body potassium calibrations for normal and obese subjects in two types of whole body counter. Phys Med Biol. 1979 Jan;24(1):171–175. doi: 10.1088/0031-9155/24/1/016. [DOI] [PubMed] [Google Scholar]
  26. Trend P. S., Wiles C. M., Spencer G. T., Morgan-Hughes J. A., Lake B. D., Patrick A. D. Acid maltase deficiency in adults. Diagnosis and management in five cases. Brain. 1985 Dec;108(Pt 4):845–860. doi: 10.1093/brain/108.4.845. [DOI] [PubMed] [Google Scholar]
  27. Umpleby A. M., Boroujerdi M. A., Brown P. M., Carson E. R., Sönksen P. H. The effect of metabolic control on leucine metabolism in type 1 (insulin-dependent) diabetic patients. Diabetologia. 1986 Mar;29(3):131–141. doi: 10.1007/BF02427082. [DOI] [PubMed] [Google Scholar]
  28. Umpleby A. M., Wiles C. M., Trend P. S., Scobie I. N., Macleod A. F., Spencer G. T., Sonksen P. H. Protein turnover in acid maltase deficiency before and after treatment with a high protein diet. J Neurol Neurosurg Psychiatry. 1987 May;50(5):587–592. doi: 10.1136/jnnp.50.5.587. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. WILLIAMSON D. H., MELLANBY J., KREBS H. A. Enzymic determination of D(-)-beta-hydroxybutyric acid and acetoacetic acid in blood. Biochem J. 1962 Jan;82:90–96. doi: 10.1042/bj0820090. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Yang R. D., Matthews D. E., Bier D. M., Lo C., Young V. R. Alanine kinetics in humans: influence of different isotopic tracers. Am J Physiol. 1984 Nov;247(5 Pt 1):E634–E638. doi: 10.1152/ajpendo.1984.247.5.E634. [DOI] [PubMed] [Google Scholar]
  31. Yang R. D., Matthews D. E., Bier D. M., Wen Z. M., Young V. R. Response of alanine metabolism in humans to manipulation of dietary protein and energy intakes. Am J Physiol. 1986 Jan;250(1 Pt 1):E39–E46. doi: 10.1152/ajpendo.1986.250.1.E39. [DOI] [PubMed] [Google Scholar]

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