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首页> 外文期刊>Molecular biology of the cell >Nitric Oxide Signaling Is Disrupted in the Yeast Model for Batten Disease
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Nitric Oxide Signaling Is Disrupted in the Yeast Model for Batten Disease

机译：一氧化碳在酵母细菌模型中的信号被破坏

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The juvenile form of neuronal ceroid lipofuscinoses (JNCLs), or Batten disease, results from mutations in the CLN3 gene, and it is characterized by the accumulation of lipopigments in the lysosomes of several cell types and by extensive neuronal death. We report that the yeast model for JNCL ( btn1 -Δ) that lacks BTN1 , the homologue to human CLN3, has increased resistance to menadione-generated oxidative stress. Expression of human CLN3 complemented the btn1 -Δ phenotype, and equivalent Btn1p/Cln3 mutations correlated with JNCL severity. We show that the previously reported decreased levels of l -arginine in btn1 -Δ limit the synthesis of nitric oxide (·NO) in both physiological and oxidative stress conditions. This defect in ·NO synthesis seems to suppress the signaling required for yeast menadione-induced apoptosis, thus explaining btn1 -Δ phenotype of increased resistance. We propose that in JNCL, a limited capacity to synthesize ·NO directly caused by the absence of Cln3 function may contribute to the pathology of the disease.
机译：幼年形式的神经元类固醇脂褐藻糖（JNCL）或Batten病是由CLN3基因突变引起的，其特征是脂质体在几种细胞类型的溶酶体中蓄积，并以广泛的神经元死亡为特征。我们报告，缺少BTN1，人类CLN3的同系物的JNCL（btn1-Δ）的酵母模型已增加了对甲萘醌产生的氧化应激的抵抗力。人CLN3的表达补充了btn1-Δ表型，等效的Btn1p / Cln3突变与JNCL严重程度相关。我们表明，先前报道的btn1-Δ中精氨酸水平的降低限制了生理和氧化应激条件下一氧化氮（·NO）的合成。 ·NO合成中的这种缺陷似乎抑制了酵母甲萘醌诱导的细胞凋亡所需的信号传导，从而解释了耐药性增加的btn1-Δ表型。我们建议在JNCL中，由于缺乏Cln3功能而直接合成·NO的能力有限，可能有助于该疾病的病理。

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