ELOVL5 (Elongase of Very-Long Fatty Acid 5) gene encodes for an enzyme that elongates long chain fatty acids, with a marked preference for polyunsaturated molecules. In particular, it plays an essential role in the elongation of omega-3 and omega-6 fatty acids, precursors for long-chain polyunsaturated fatty acids (PUFAs). Mutations of ELOVL5 cause the spino-cerebellar ataxia type 38 (SCA38), a rare autosomal neurological disease characterized by gait abnormality, dysarthria, dysphagia, hyposmia and peripheral neuropathy, conditions well represented by a mouse model with a targeted deletion of this gene (Elovl5-/- mice). However, the expression pattern of this enzyme in neuronal and glial cells of the central nervous system (CNS) is still uninvestigated. This work is aimed at filling this gap of knowledge by taking advantage of an Elovl5-reporter mouse line and immunofluorescence analyses on adult mouse CNS sections and glial cell primary cultures. Notably, Elovl5 appears expressed in a region- and cell type-specific manner. Abundant Elovl5-positive cells were found in the cerebellum, brainstem, and primary and accessory olfactory regions, where mitral cells show the most prominent expression. Hippocampal pyramidal cells of CA2/CA3 where also moderately labeled, while in the rest of the telencephalons Elovl5 expression was high in regions related to motor control. Analysis of primary glial cell cultures revealed Elovl5 expression in oligodendroglial cells at various maturation steps and in microglia, while astrocytes showed a heterogeneous in vivo expression of Elovl5. The elucidation of Elovl5 CNS distribution provides relevant information to understand the physiological functions of this enzyme and its PUFA products, whose unbalance is known to be involved in many pathological conditions.

中文翻译：

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Elovl5在成年小鼠中枢神经系统中的表达

ELOVL5（极长脂肪酸5的延伸酶）基因编码一种酶，该酶可延长长链脂肪酸的含量，特别是对多不饱和分子的偏爱。特别地，它在伸长长链多不饱和脂肪酸（PUFA）的omega-3和omega-6脂肪酸中起着至关重要的作用。ELOVL5突变会导致38型脊髓小脑共济失调（SCA38），这是一种罕见的常染色体神经系统疾病，其特征是步态异常，构音障碍，吞咽困难，体力低下和周围神经病变，其病情好于具有该基因靶向缺失的小鼠模型（Elovl5 -/- 老鼠）。但是，该酶在中枢神经系统（CNS）的神经元和神经胶质细胞中的表达模式仍待研究。这项工作旨在通过利用Elovl5报告基因小鼠品系以及对成年小鼠CNS切片和神经胶质细胞原代培养物进行免疫荧光分析来填补这一知识空白。值得注意的是，Elovl5似乎以区域和细胞类型特定的方式表达。在小脑，脑干以及主要和辅助嗅觉区域发现了丰富的Elovl5阳性细胞，其中二尖瓣细胞显示出最突出的表达。CA2 / CA3的海马锥体细胞也被适度地标记，而在端脑的其余部分，Elov15在与运动控制有关的区域中的表达较高。对原代神经胶质细胞培养物的分析显示，在不同成熟步骤的少突胶质细胞和小胶质细胞中，Elovl5的表达，而星形胶质细胞则在体内表达了Elovl5的异质性。