Goldstein David S, Sullivan Patti, Holmes Courtney
Autonomic Medicine Section (AMS), Clinical Neurosciences Program (CNP), Division of Intramural Research (DIR), National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), 10 Center Drive MSC-1620, Building 10 Room 8N260, Bethesda, MD, 20892-1620, USA.
Clin Auton Res. 2025 Apr;35(2):215-222. doi: 10.1007/s10286-024-01093-6. Epub 2024 Dec 10.
Autonomic synucleinopathies feature autonomic failure and intracellular deposition of the protein alpha-synuclein. Three such conditions are the Lewy body diseases (LBDs) Parkinson's disease (PD) and pure autonomic failure (PAF) and the non-LBD synucleinopathy multiple system atrophy (MSA). These diseases all entail catecholaminergic abnormalities in the brain, sympathetically innervated organs, or both; however, little is known about renal catecholaminergic functions in autonomic synucleinopathies. We measured urinary excretion rates of the sympathetic neurotransmitter norepinephrine, the hormone epinephrine, the autocrine-paracrine substance dopamine, the catecholamine precursor 3,4-dihydroxyphenylalanine (DOPA), 3,4-dihydroxyphenylglycol (DHPG, the main neuronal metabolite of norepinephrine), and 3,4-dihydroxyphenylacetic acid (DOPAC, a major dopamine metabolite), in PD, PAF, and MSA groups and controls.
Data were reviewed from all research participants who had urine collections (usually 3.5 h) at the National Institutes of Health (NIH) Clinical Center from 1995 to 2024. The control cohort had neither autonomic failure nor a movement disorder.
Norepinephrine excretion rates were decreased compared with controls in PD (p = 0.0001), PAF (p < 0.0001), and MSA (p < 0.0001). Dopamine excretion was also decreased in the three groups (PD: p = 0.0136, PAF: p = 0.0027, MSA: p = 0.0344). DHPG excretion was decreased in PD (p = 0.0004) and PAF (p = 0.0004) but not in MSA. DOPA and epinephrine excretion did not differ among the study groups.
Autonomic synucleinopathies involve decreased urinary excretion rates of norepinephrine and dopamine. Since virtually all of urinary dopamine in humans is derived from circulating DOPA, the low rates of urinary norepinephrine and dopamine excretion may reflect dysfunctions in the renal sympathetic noradrenergic system, the DOPA-dopamine autocrine-paracrine system, or both systems.
自主神经α-突触核蛋白病的特征是自主神经功能衰竭和α-突触核蛋白在细胞内沉积。三种此类病症为路易体病(LBDs)、帕金森病(PD)和纯自主神经功能衰竭(PAF)以及非LBD突触核蛋白病多系统萎缩(MSA)。这些疾病均伴有大脑、交感神经支配的器官或两者中的儿茶酚胺能异常;然而,关于自主神经突触核蛋白病中肾儿茶酚胺能功能知之甚少。我们测量了PD、PAF和MSA组及对照组中交感神经递质去甲肾上腺素、激素肾上腺素、自分泌-旁分泌物质多巴胺、儿茶酚胺前体3,4-二羟基苯丙氨酸(DOPA)、3,4-二羟基苯乙二醇(DHPG,去甲肾上腺素的主要神经元代谢产物)和3,4-二羟基苯乙酸(DOPAC,一种主要的多巴胺代谢产物)的尿排泄率。
回顾了1995年至2024年在美国国立卫生研究院(NIH)临床中心进行尿液收集(通常为3.5小时)的所有研究参与者的数据。对照组既无自主神经功能衰竭也无运动障碍。
与对照组相比,PD组(p = 0.0001)、PAF组(p < 0.0001)和MSA组(p < 0.0001)的去甲肾上腺素排泄率降低。三组的多巴胺排泄也降低(PD组:p = 0.0136,PAF组:p = 0.0027,MSA组:p = 0.0344)。PD组(p = 0.0004)和PAF组(p = 0.0004)的DHPG排泄降低,但MSA组未降低。DOPA和肾上腺素排泄在各研究组之间无差异。
自主神经突触核蛋白病涉及去甲肾上腺素和多巴胺尿排泄率降低。由于人类尿液中的多巴胺几乎全部来源于循环中的DOPA,去甲肾上腺素和多巴胺尿排泄率低可能反映肾交感去甲肾上腺素能系统、DOPA-多巴胺自分泌-旁分泌系统或这两个系统的功能障碍。