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Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis

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References

  1. Das AM, Byrd DJ, Brodehl J (1994) Regulation of the mitochondrial ATPsynthase in human fibroblasts.Clin Chim Acta 231: 61–68.

  2. Ezaki J, Wolfe LS, Ishido K, Kominami E (1995) Abnormal degradative pathway of mitochondrial ATP synthase subunitc in late infantile neuronal ceroid lipofuscinosis (Batten disease).Am J Med Genet 57: 254–259.

  3. Hall NA, Lake BD, Dewij NN, Patrick AD (1991) Lysosomal storage of subunitc of mitochondrial ATPsynthase in Batten's disease.Biochem J 275: 269–272.

  4. Kohlschütter A, Gardiner RM, Goebel HH (1993) Human forms of neuronal ceroid lipofuscinosis (Batten disease): consensus diagnostic criteria, Hamburg 1992.J Inher Metab Dis 16: 241–244.

  5. Lippe G, Sorgato MC, Harris DA (1988) The binding of the inhibitor protein is governed independently by ATP and membrane potential in ox-heart submitochondrial particles.Biochim Biophys Acta 933: 12–21.

  6. Stumpf DA, Parks JK (1981) Human mitochondrial electron transport chain: assay of succinate: cytochromec reductase in leukocytes, platelets and cultured fibroblasts.Biochem Med 25: 234–238.

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Correspondence to A. M. Das.

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Das, A.M., Kohlschütter, A. Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis. J Inherit Metab Dis 19, 130–132 (1996). https://doi.org/10.1007/BF01799411

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Keywords

  • Public Health
  • Internal Medicine
  • Metabolic Disease
  • Neuronal Ceroid Lipofuscinosis
  • Juvenile Neuronal Ceroid Lipofuscinosis