Background

Telomeres are repetitive DNA sequences of TTAGGG at the ends of chromosomes. Many studies have shown that telomere shortening is associated with aging-related diseases, such as cardiovascular diseases, hypertension, diabetes, cancer, and various neurodegenerative diseases, including Alzheimer's disease, vascular dementia, Parkinson's disease, and dementia with Lewy bodies. However, changes in telomere length (TL) in patients with frontotemporal dementia (FTD) syndrome are unclear. Accordingly, in this study, we assessed TL in blood samples from patients with FTD syndrome.

Methods

Absolute TL was measured in peripheral blood leukocytes from 53 patients with FTD syndromes (25 with behavioral variant FTD, 19 with semantic variant primary progressive aphasia [PPA], six with nonfluent/agrammatic variant PPA, and three with amyotrophic lateral sclerosis [ALS] plus) and 28 cognitively unimpaired (CU) controls using terminal restriction fragment analysis.

Results

TL was significantly longer in the FTD group than in the CU group. All FTD subtypes had significantly longer TL than controls. There were no significant differences in TL among FTD syndromes. No significant correlations were found between TL and demographic factors in the FTD group.

Conclusions

Longer telomeres were associated with FTD syndrome, consistent with a recent report demonstrating that longer telomeres are related to ALS. Therefore, our results may support a shared biology between FTD and ALS. More studies with larger sample sizes are needed.

中文翻译：

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额颞叶痴呆综合征患者端粒长度增加

背景

端粒是染色体末端 TTAGGG 的重复 DNA 序列。许多研究表明，端粒缩短与衰老相关疾病有关，例如心血管疾病、高血压、糖尿病、癌症以及各种神经退行性疾病，包括阿尔茨海默病、血管性痴呆、帕金森病和路易体痴呆。然而，额颞叶痴呆 (FTD) 综合征患者端粒长度 (TL) 的变化尚不清楚。因此，在本研究中，我们评估了 FTD 综合征患者血液样本中的 TL。

方法

在来自 53 名 FTD 综合征患者（25 名具有行为变异 FTD，19 名具有语义变异原发性进行性失语 [PPA]，6 名具有非流利/无语法变异 PPA，3 名患有肌萎缩侧索硬化 [ALS] 加上) 和使用终端限制片段分析的 28 个认知未受损 (CU) 对照。

结果

FTD 组的 TL 显着长于 CU 组。所有 FTD 亚型的 TL 显着长于对照。FTD 综合征之间的 TL 没有显着差异。在 FTD 组中，TL 与人口统计学因素之间未发现显着相关性。

结论

较长的端粒与 FTD 综合征相关，这与最近的一份报告一致，表明较长的端粒与 ALS 相关。因此，我们的结果可能支持 FTD 和 ALS 之间的共享生物学。需要更多样本量更大的研究。