BTN1, a yeast gene corresponding to the human gene responsible for Batten's disease, is not essential for viability, mitochondrial function, or degradation of mitochondrial ATP synthase

Yeast

Volumn 13, Issue 8, 1997, Pages 691-697

  Pearce, David A ^a   Sherman, Fred ^a  

^a UNIVERSITY OF ROCHESTER   (United States)

Author keywords

ATP synthase; Batten's disease; Degradation; Mitochondria; Saccharomyces cerevisiae

Indexed keywords

PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

ARTICLE; ENZYME DEGRADATION; FUNGAL GENETICS; GENE EXPRESSION; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PRIORITY JOURNAL; SACCHAROMYCES CEREVISIAE; SEQUENCE HOMOLOGY;

AMINO ACID SEQUENCE; CYCLINS; FUNGAL PROTEINS; GENES, FUNGAL; HUMANS; MEMBRANE GLYCOPROTEINS; MITOCHONDRIA; MOLECULAR CHAPERONES; MOLECULAR SEQUENCE DATA; NEURONAL CEROID-LIPOFUSCINOSES; PROTON-TRANSLOCATING ATPASES; SACCHAROMYCES CEREVISIAE; SACCHAROMYCES CEREVISIAE PROTEINS;

SACCHAROMYCES CEREVISIAE;

EID: 0030855171     PISSN: 0749503X     EISSN: None     Source Type: Journal    
DOI: 10.1002/(SICI)1097-0061(19970630)13:8<691::AID-YEA123>3.0.CO;2-D     Document Type: Article

References (25)

1. Banerjee, P., Dasgupta, A., Siakotos, A. N. and Dawson, G. (1992). Evidence for lipase abnormality, high levels of free and triacylglycerol forms of unsaturated fatty acids in neuronal ceroid-lipofuscinosis tissue. Am. J. Med. Genet. 42, 549-554.
2. Ezaki, J., Wolfe, L. S., Ishidoh, K. and Kominami, E. (1995b). Abnormal degradative pathway of mitochondrial atp synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten Disease). Am. J. Med. Genet. 57, 254-259.
3. Ezaki, J., Wolfe, L. S., Higuiti, T., Ishidoh, K. and Kominami, E. (1995a). Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten Disease). J. Neurochem. 64, 733-741.
4. Goebel, H. H. (1995). The neuronal ceroid-lipofuscinoses. J. Child. Neurol. 10, 424-437.
5. Guelin, E., Rep, M. and Grivell, L. A. (1994). Sequence of the AFG3 gene encoding a new member of the Fts/Yme1/Tma subfamily of the AAA-protein family. Yeast 10, 1389-1394.
6. Guelin, E., Rep, M. and Grivell, L. A. (1996). Afg3p, a mitochondrial ATP-dependent metalloprotease, is involved in degradation of mitochondrial-encoded Cox1, Cox3, Cob, Su6, Su8 and Su9 subunits of the inner membrane complexes III, IV and V. FEBS Lett. 381, 42-46.
7. Hall, N. A., Lake, B. D., Dewji, N. N. and Patrick, A. D. (1991). Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis). Biochem. J. 275, 269-272.
8. Haltia, M., Rapola, L., Santavuori, P. and Keranen, A. (1973). Infantile type of so-called neuronal ceroid-lipofuscinosis. 2. Morphological and biochemical studies. J. Neurol. Sci. 18, 269-285.
9. Kominami, E., Ezaki, J., Muno, D., Ishido, K., Ueno, T. and Wolfe, L. S. (1992). Specific storage of subunit c of mitochondrial atp synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's Disease). J. Biochem. 111, 278-282.
10. Lee, R. L., Johnson, K. R. and Lerner, T. J. (1996). Isolation and chromosomal mapping of a mouse homolog of the Batten disease gene CLN3. Genomics 35, 617-619.
11. Nakai, T., Yasuhara, T., Fujiki, Y. and Ohashi, A. (1995). Multiple genes, including a member of the AA family, are essential for degradation of unassembled subunit 2 of cytochrome c oxidase in yeast mitochondria. Mol. Cell. Biol. 15, 4441-4452.
12. Palmer, D. N., Bayliss, S. L. and Westlake, V. J. (1995). Batten disease and the ATP synthase subunit c turnover pathway, raising antibodies to subunit c. Am. J. Med. Genet. 57, 260-265.
13. 1-ATPase assembly in Saccharomyces cerevisiae. FEBS Lett. 373, 66-70.
14. Pearce, D. A. and Sherman, F. (1995a). Diminished degradation of yeast cytochrome c by interactions with its physiological partners. Proc. Natl. Acad. Sci. USA 92, 3735-3739.
15. Pearce, D. A. and Sherman, F. (1995b). Degradation of cytochrome oxidase subunits in mutants of yeast lacking cytochrome c and suppression of the degradation by mutation of yme1. J. Biol. Chem. 270, 20879-20882.
16. Reid, G. (1983). Pulse labeling of yeast cells and spheroplasts. Methods Enzymol. 97, 324-329.
17. Schagger, H. and von Jagow, G. (1987). Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa. Anal. Biochem. 16, 368-379.
18. Sherman, F. (1991). Getting started with yeast. Methods Enzymol. 194, 3-21.
19. Suzuki, C. K., Suda, K., Wang, N. and Schatz, G. (1994). Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration. Science 264, 273-276.
20. The International Batten Disease Consortium. (1995). Isolation of a novel gene underlying Batten disease, CLN3. Cell 82, 949-957.
21. Thorsness, P. E., White, K. H. and Fox, T. D. (1993). Inactivation of YME1, a member of the fstH-SEC18-PAS1-CDC48 family of putative ATPase-encoding genes, causes increased escape of DNA from mitochondria in Saccharomyces cerevisiae. Mol. Cell. Biol. 13, 5418-5426.
22. Tzagoloff, A., Yue, J., Jang, J. and Paul, M. F. (1994). A new member of a family of ATPases is essential for assembly of mitochondrial respiratory chain and ATP synthetase complexes in Saccharomyces cerevisiae. J. Biol. Chem. 269, 26144-26151.
23. Van Dyck, L., Pearce, D. A. and Sherman, F. (1994). PIM1 encodes a mitochondrial ATP-dependent protease that is required for mitochondrial function in the yeast Saccharomyces cerevisiae. J. Biol. Chem. 269, 238-242.
24. Wagner, I., Arlt, H., Van Dyck, L., Langer, T. and Neupert, W. (1994). Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria. EMBO J. 13, 5135-5145.
25. Yaffe, M. P. and Schatz, G. (1984). Two nuclear mutations that block mitochondrial protein import in yeast. Proc. Natl. Acad. Sci. USA 81, 4819-4823.