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Journal of Bacteriology, November 2000, p. 6418-6423, Vol. 182, No. 22
0021-9193/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.

The Yeast Model for Batten Disease: Mutations in btn1, btn2, and hsp30 Alter pH Homeostasis

Subrata Chattopadhyay,1 Neda E. Muzaffar,1 Fred Sherman,2 and David A. Pearce1,2,*

Center for Aging and Developmental Biology1 and the Department of Biochemistry and Biophysics,2 University of Rochester Medical School, Rochester, New York 14642

Received 29 March 2000/Accepted 22 August 2000

The BTN1 gene product of the yeast Saccharomyces cerevisiae is 39% identical and 59% similar to human CLN3, which is associated with the neurodegenerative disorder Batten disease. Furthermore, btn1-Delta strains have an elevated activity of the plasma membrane H+-ATPase due to an abnormally high vacuolar acidity during the early phase of growth. Previously, DNA microarray analysis revealed that btn1-Delta strains compensate for the altered plasma membrane H+-ATPase activity and vacuolar pH by elevating the expression of the two genes HSP30 and BTN2. We now show that deletion of either HSP30 or BTN2 in either BTN1+ or btn1-Delta strains does not alter vacuolar pH but does lead to an increased activity of the vacuolar H+-ATPase. Deletion of BTN1, BTN2, or HSP30 does not alter cytosolic pH but diminishes pH buffering capacity and causes poor growth at low pH in a medium containing sorbic acid, a condition known to result in disturbed intracellular pH homeostasis. Btn2p was localized to the cytosol, suggesting a role in mediating pH homeostasis between the vacuole and plasma membrane H+-ATPase. Increased expression of HSP30 and BTN2 in btn1-Delta strains and diminished growth of btn1-Delta , hsp30-Delta , and btn2-Delta strains at low pH reinforce our view that altered pH homeostasis is the underlying cause of Batten disease.

* Corresponding author. Mailing address: Center for Aging and Developmental Biology, University of Rochester School of Medicine, 601 Elmwood Ave., Rochester, NY 14642. Phone: (716) 273-1514. Fax: (716) 756-7665. E-mail: David_Pearce{at}urmc.rochester.edu.

Journal of Bacteriology, November 2000, p. 6418-6423, Vol. 182, No. 22
0021-9193/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.


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