Pearce D A, Nosel S A, Sherman F
Department of Biochemistry and Biophysics, University of Rochester School of Medicine and Dentistry, Rochester, New York 14642, USA.
Mol Genet Metab. 1999 Apr;66(4):320-3. doi: 10.1006/mgme.1999.2819.
Although the gene responsible for Batten disease, CLN3, was positionally cloned in 1995, the function of Cln3p and the molecular basis of the disease still remain elusive. We previously reported that the yeast Saccharomyces cerevisiae contains a homolog to Cln3p, designated Btn1p, and that the human Cln3p complemented the pH-dependent resistance to D-(-)-threo-2-amino-1-[p-nitrophenyl]-1, 3-propanediol in btn1-Delta yeast mutants. We have determined that yeast lacking Btn1p have an elevated ability to acidify media during growth that correlates with an elevated plasma membrane ATPase activity. Btn1p may be involved in maintaining pH homeostasis of yeast cells.
尽管导致巴滕病的基因CLN3在1995年通过定位克隆得到,但Cln3p的功能以及该疾病的分子基础仍然难以捉摸。我们之前报道过,酿酒酵母含有一个与Cln3p同源的蛋白,命名为Btn1p,并且人Cln3p可弥补btn1-Δ酵母突变体中对D-(-)-苏式-2-氨基-1-[对硝基苯基]-1,3-丙二醇的pH依赖性抗性。我们已经确定,缺乏Btn1p的酵母在生长过程中酸化培养基的能力增强,这与质膜ATP酶活性升高相关。Btn1p可能参与维持酵母细胞的pH稳态。