Division of Endocrinology, Fundación Jiménez Díaz, 28040, Madrid, Spain.
Department of Neurology, Movement Disorders Unit, Fundación Jiménez Díaz, 28040, Madrid, Spain.
Mol Nutr Food Res. 2018 Dec;62(23):e1800619. doi: 10.1002/mnfr.201800619. Epub 2018 Nov 8.
Huntington's disease (HD) is a rare progressive neurodegenerative disorder of genetic origin, with no definitive treatment. Unintentional weight loss (UWL) is a clinical feature of symptomatic HD subjects. To prevent UWL, a customized HD diet is designed and its impact on plasma miRNA HD footprint and neurological parameters is examined.
Eleven participants are included, BMI ≤ 18 kg m or UWL of 5% in 6 months or 10% in a year. Diet design is based on nutritional surveys and interviews of participants and caregivers and on published literature review. Twelve-month dietary intervention, with follow-up every 3 months, induces high diet adherence, which manages to curb UWL in all participants (73% gained weight). Noticeable increases in fat mass and leptin levels are obtained. The results also show significant decrease in the expression of 19 miRNAs, which are previously reported to be upregulated in HD-patients versus healthy controls: revealing hsa-miR-338-3p, hsa-miR-128-3p, hsa-miR-23a-3p, and hsa-miR-24-3p as potential HD-biomarkers. The diminished expression of hsa-miR-100-5p reflects the general maintenance of the functional status. Cognitive status is improved in six of 11 participants, while only three present better motor-score values.
A customized HD-diet prevents UWL and modified miRNAs HD-footprint. The normalization of miRNA values suggests its potentially use as HD-biomarkers.
亨廷顿病(HD)是一种罕见的进行性神经退行性遗传疾病,目前尚无明确的治疗方法。非故意体重减轻(UWL)是有症状的 HD 患者的临床特征。为了预防 UWL,设计了一种定制的 HD 饮食,并检查了其对血浆 miRNA HD 特征和神经参数的影响。
纳入 11 名参与者,BMI≤18kg/m 或 6 个月内 UWL 为 5%,或 1 年内 UWL 为 10%。饮食设计基于参与者和护理人员的营养调查和访谈,以及已发表的文献综述。为期 12 个月的饮食干预,每 3 个月随访一次,可实现高度的饮食依从性,成功遏制了所有参与者的 UWL(73%的人体重增加)。可观察到体脂和瘦素水平显著增加。结果还表明,19 种 miRNA 的表达显著下降,这些 miRNA 之前被报道在 HD 患者与健康对照组中上调:表明 hsa-miR-338-3p、hsa-miR-128-3p、hsa-miR-23a-3p 和 hsa-miR-24-3p 可能是 HD 的生物标志物。hsa-miR-100-5p 的表达减少反映了功能状态的普遍维持。11 名参与者中有 6 名认知状态得到改善,而只有 3 名运动评分值更好。
定制的 HD 饮食可预防 UWL 和改变的 miRNA HD 特征。miRNA 值的正常化表明其可能作为 HD 生物标志物的用途。