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178 related items for PubMed ID: 8858953

  • 1. Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c.
    Ezaki J, Wolfe LS, Kominami E.
    J Neurochem; 1996 Oct; 67(4):1677-87. PubMed ID: 8858953
    [Abstract] [Full Text] [Related]

  • 2. Tripeptidyl peptidase I, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase.
    Ezaki J, Takeda-Ezaki M, Kominami E.
    J Biochem; 2000 Sep; 128(3):509-16. PubMed ID: 10965052
    [Abstract] [Full Text] [Related]

  • 3. Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten disease).
    Ezaki J, Wolfe LS, Ishidoh K, Kominami E.
    Am J Med Genet; 1995 Jun 05; 57(2):254-9. PubMed ID: 7668341
    [Abstract] [Full Text] [Related]

  • 4. Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease).
    Ezaki J, Wolfe LS, Higuti T, Ishidoh K, Kominami E.
    J Neurochem; 1995 Feb 05; 64(2):733-41. PubMed ID: 7830067
    [Abstract] [Full Text] [Related]

  • 5. A lysosomal proteinase, the late infantile neuronal ceroid lipofuscinosis gene (CLN2) product, is essential for degradation of a hydrophobic protein, the subunit c of ATP synthase.
    Ezaki J, Tanida I, Kanehagi N, Kominami E.
    J Neurochem; 1999 Jun 05; 72(6):2573-82. PubMed ID: 10349869
    [Abstract] [Full Text] [Related]

  • 6. Specific storage of subunit c of mitochondrial ATP synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's disease).
    Kominami E, Ezaki J, Muno D, Ishido K, Ueno T, Wolfe LS.
    J Biochem; 1992 Feb 05; 111(2):278-82. PubMed ID: 1533218
    [Abstract] [Full Text] [Related]

  • 7. Decreased lysosomal subunit c-degrading activity in fibroblasts from patients with late infantile neuronal ceroid lipofuscinosis.
    Ezaki J, Wolfe LS, Kominami E.
    Neuropediatrics; 1997 Feb 05; 28(1):53-5. PubMed ID: 9151323
    [Abstract] [Full Text] [Related]

  • 8. New insight into lysosomal protein storage disease: delayed catabolism of ATP synthase subunit c in Batten disease.
    Kominami E, Ezaki J, Wolfe LS.
    Neurochem Res; 1995 Nov 05; 20(11):1305-9. PubMed ID: 8786816
    [Abstract] [Full Text] [Related]

  • 9. Anomalies of mitochondrial ATP synthase regulation in four different types of neuronal ceroid lipofuscinosis.
    Das AM, Jolly RD, Kohlschütter A.
    Mol Genet Metab; 1999 Apr 05; 66(4):349-55. PubMed ID: 10191128
    [Abstract] [Full Text] [Related]

  • 10. Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.
    Palmer DN, Fearnley IM, Medd SM, Walker JE, Martinus RD, Bayliss SL, Hall NA, Lake BD, Wolfe LS, Jolly RD.
    Adv Exp Med Biol; 1989 Apr 05; 266():211-22; discussion 223. PubMed ID: 2535017
    [Abstract] [Full Text] [Related]

  • 11. [Batten disease (Neuronal ceroid lipofuscinoses)--accumulation of ATP synthase subunit c caused by the delay of lysosomal degradation].
    Ezaki J, Kominami E.
    Nihon Rinsho; 1995 Dec 05; 53(12):3055-61. PubMed ID: 8577058
    [Abstract] [Full Text] [Related]

  • 12. Turnover of F1F0-ATP synthase subunit 9 and other proteolipids in normal and Batten disease fibroblasts.
    Tanner A, Shen BH, Dice JF.
    Biochim Biophys Acta; 1997 Oct 24; 1361(3):251-62. PubMed ID: 9375799
    [Abstract] [Full Text] [Related]

  • 13. The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase.
    Fearnley IM, Walker JE, Martinus RD, Jolly RD, Kirkland KB, Shaw GJ, Palmer DN.
    Biochem J; 1990 Jun 15; 268(3):751-8. PubMed ID: 2141977
    [Abstract] [Full Text] [Related]

  • 14. Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease).
    Palmer DN, Fearnley IM, Walker JE, Hall NA, Lake BD, Wolfe LS, Haltia M, Martinus RD, Jolly RD.
    Am J Med Genet; 1992 Feb 15; 42(4):561-7. PubMed ID: 1535179
    [Abstract] [Full Text] [Related]

  • 15. Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).
    Hall NA, Lake BD, Dewji NN, Patrick AD.
    Biochem J; 1991 Apr 01; 275 ( Pt 1)(Pt 1):269-72. PubMed ID: 1826833
    [Abstract] [Full Text] [Related]

  • 16. Defect of proteolysis of mitochondrial ATP synthase subunit C in neuronal ceroid lipofuscinosis.
    Ezaki J, Wolfe LS, Kominami E.
    Gerontology; 1995 Apr 01; 41 Suppl 2():259-69. PubMed ID: 8821337
    [Abstract] [Full Text] [Related]

  • 17. Late-infantile ceroid-lipofuscinosis: lysine methylation of mitochondrial ATP synthase subunit c from lysosomal storage bodies.
    Katz ML, Siakotos AN, Gao Q, Freiha B, Chin DT.
    Biochim Biophys Acta; 1997 Jul 10; 1361(1):66-74. PubMed ID: 9247091
    [Abstract] [Full Text] [Related]

  • 18. Enzyme replacement therapy with recombinant pro-CTSD (cathepsin D) corrects defective proteolysis and autophagy in neuronal ceroid lipofuscinosis.
    Marques ARA, Di Spiezio A, Thießen N, Schmidt L, Grötzinger J, Lüllmann-Rauch R, Damme M, Storck SE, Pietrzik CU, Fogh J, Bär J, Mikhaylova M, Glatzel M, Bassal M, Bartsch U, Saftig P.
    Autophagy; 2020 May 10; 16(5):811-825. PubMed ID: 31282275
    [Abstract] [Full Text] [Related]

  • 19. Lysine methylation of mitochondrial ATP synthase subunit c stored in tissues of dogs with hereditary ceroid lipofuscinosis.
    Katz ML, Christianson JS, Norbury NE, Gao CL, Siakotos AN, Koppang N.
    J Biol Chem; 1994 Apr 01; 269(13):9906-11. PubMed ID: 8144584
    [Abstract] [Full Text] [Related]

  • 20. Molecular basis of lysosomal accumulation of subunit c of mitochondrial ATP synthase in neuronal ceroid-lipofuscinosis.
    Ezaki J, Wolfe LS, Kominami E.
    J Inherit Metab Dis; 1993 Apr 01; 16(2):296-8. PubMed ID: 8411985
    [No Abstract] [Full Text] [Related]

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