These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
129 related articles for article (PubMed ID: 8411985)
1. 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; 16(2):296-8. PubMed ID: 8411985 [No Abstract] [Full Text] [Related]
2. 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; 275 ( Pt 1)(Pt 1):269-72. PubMed ID: 1826833 [TBL] [Abstract][Full Text] [Related]
3. 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; 111(2):278-82. PubMed ID: 1533218 [TBL] [Abstract][Full Text] [Related]
4. 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 [TBL] [Abstract][Full Text] [Related]
5. 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; 266():211-22; discussion 223. PubMed ID: 2535017 [TBL] [Abstract][Full Text] [Related]
6. 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 [TBL] [Abstract][Full Text] [Related]
7. 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; 64(2):733-41. PubMed ID: 7830067 [TBL] [Abstract][Full Text] [Related]
8. 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; 57(2):254-9. PubMed ID: 7668341 [TBL] [Abstract][Full Text] [Related]
9. 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; 42(4):561-7. PubMed ID: 1535179 [TBL] [Abstract][Full Text] [Related]
10. 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; 269(13):9906-11. PubMed ID: 8144584 [TBL] [Abstract][Full Text] [Related]
11. Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis. Das AM; Kohlschütter A J Inherit Metab Dis; 1996; 19(2):130-2. PubMed ID: 8739947 [No Abstract] [Full Text] [Related]
12. Increased expression of subunit c of mitochondrial ATP synthase in brain tissue from neuronal ceroid lipofuscinoses and mucopolysaccharidosis cases but not in long-term fibroblast cultures. Kida E; Wisniewski KE; Golabek AA Neurosci Lett; 1993 Dec; 164(1-2):121-4. PubMed ID: 8152585 [TBL] [Abstract][Full Text] [Related]
13. 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; 72(6):2573-82. PubMed ID: 10349869 [TBL] [Abstract][Full Text] [Related]
15. Accumulation of mitochondrial ATP synthase subunit c in muscle in a patient with neuronal ceroid lipofuscinosis (late infantile form). Umehara F; Higuchi I; Tanaka K; Niiyama T; Ezaki J; Kominami E; Osame M Acta Neuropathol; 1997 Jun; 93(6):628-32. PubMed ID: 9194903 [TBL] [Abstract][Full Text] [Related]
16. Lysosomal Storage of Subunit c of Mitochondrial ATP Synthase in Brain-Specific Atp13a2-Deficient Mice. Sato S; Koike M; Funayama M; Ezaki J; Fukuda T; Ueno T; Uchiyama Y; Hattori N Am J Pathol; 2016 Dec; 186(12):3074-3082. PubMed ID: 27770614 [TBL] [Abstract][Full Text] [Related]
17. Bovine ceroid-lipofuscinosis (Batten's disease): the major component stored is the DCCD-reactive proteolipid, subunit C, of mitochondrial ATP synthase. Martinus RD; Harper PA; Jolly RD; Bayliss SL; Midwinter GG; Shaw GJ; Palmer DN Vet Res Commun; 1991; 15(2):85-94. PubMed ID: 1829867 [TBL] [Abstract][Full Text] [Related]
18. 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; 28(1):53-5. PubMed ID: 9151323 [TBL] [Abstract][Full Text] [Related]
19. Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons. Koike M; Nakanishi H; Saftig P; Ezaki J; Isahara K; Ohsawa Y; Schulz-Schaeffer W; Watanabe T; Waguri S; Kametaka S; Shibata M; Yamamoto K; Kominami E; Peters C; von Figura K; Uchiyama Y J Neurosci; 2000 Sep; 20(18):6898-906. PubMed ID: 10995834 [TBL] [Abstract][Full Text] [Related]
20. Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis. Medd SM; Walker JE; Jolly RD Biochem J; 1993 Jul; 293 ( Pt 1)(Pt 1):65-73. PubMed ID: 8328973 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]