Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease

Objective: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. Neurofilament light protein (NfL) is correlated with clinical severity of HD but relative data are the lack in the Chinese population. Reactive astrocytes are related to HD pathology, which predicts their...

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Autores principales:	Huajing You, Tengteng Wu, Gang Du, Yue Huang, Yixuan Zeng, Lishan Lin, Dingbang Chen, Chao Wu, Xunhua Li, Jean-marc Burgunder, Zhong Pei
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	Huntington's disease glial fibrillary acidic protein neurofilament light protein clinical severity biomarker Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/7597e9b8b9594d0e9cd79cb62e077540
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spelling	oai:doaj.org-article:7597e9b8b9594d0e9cd79cb62e0775402021-11-19T05:12:31ZEvaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease1664-229510.3389/fneur.2021.779890https://doaj.org/article/7597e9b8b9594d0e9cd79cb62e0775402021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fneur.2021.779890/fullhttps://doaj.org/toc/1664-2295Objective: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. Neurofilament light protein (NfL) is correlated with clinical severity of HD but relative data are the lack in the Chinese population. Reactive astrocytes are related to HD pathology, which predicts their potential to be a biomarker in HD progression. Our aim was to discuss the role of blood glial fibrillary acidic protein (GFAP) to evaluate clinical severity in patients with HD.Methods: Fifty-seven HD mutation carriers (15 premanifest HD, preHD, and 42 manifest HD) and 26 healthy controls were recruited. Demographic data and clinical severity assessed with the internationally Unified Huntington's Disease Rating Scale (UHDRS) were retrospectively analyzed. Plasma NfL and GFAP were quantified with an ultra-sensitive single-molecule (Simoa, Norcross, GA, USA) technology. We explored their consistency and their correlation with clinical severity.Results: Compared with healthy controls, plasma NfL (p < 0.0001) and GFAP (p < 0.001) were increased in Chinese HD mutation carriers, and they were linearly correlated with each other (r = 0.612, p < 0.001). They were also significantly correlated with disease burden, Total Motor Score (TMS) and Total Functional Capacity (TFC). The scores of Stroop word reading, symbol digit modalities tests, and short version of the Problem Behaviors Assessments (PBAs) for HD were correlated with plasma NfL but not GFAP. Compared with healthy controls, plasma NfL has been increased since stage 1 but plasma GFAP began to increase statistically in stage 2.Conclusions: Plasma GFAP was correlated with plasma NfL, disease burden, TMS, and TFC in HD mutation carriers. Plasma GFAP may have potential to be a sensitive biomarker for evaluating HD progression.Huajing YouTengteng WuGang DuGang DuYue HuangYue HuangYixuan ZengLishan LinDingbang ChenChao WuXunhua LiJean-marc BurgunderZhong PeiFrontiers Media S.A.articleHuntington's diseaseglial fibrillary acidic proteinneurofilament light proteinclinical severitybiomarkerNeurology. Diseases of the nervous systemRC346-429ENFrontiers in Neurology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Huntington's disease glial fibrillary acidic protein neurofilament light protein clinical severity biomarker Neurology. Diseases of the nervous system RC346-429
spellingShingle	Huntington's disease glial fibrillary acidic protein neurofilament light protein clinical severity biomarker Neurology. Diseases of the nervous system RC346-429 Huajing You Tengteng Wu Gang Du Gang Du Yue Huang Yue Huang Yixuan Zeng Lishan Lin Dingbang Chen Chao Wu Xunhua Li Jean-marc Burgunder Zhong Pei Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
description	Objective: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. Neurofilament light protein (NfL) is correlated with clinical severity of HD but relative data are the lack in the Chinese population. Reactive astrocytes are related to HD pathology, which predicts their potential to be a biomarker in HD progression. Our aim was to discuss the role of blood glial fibrillary acidic protein (GFAP) to evaluate clinical severity in patients with HD.Methods: Fifty-seven HD mutation carriers (15 premanifest HD, preHD, and 42 manifest HD) and 26 healthy controls were recruited. Demographic data and clinical severity assessed with the internationally Unified Huntington's Disease Rating Scale (UHDRS) were retrospectively analyzed. Plasma NfL and GFAP were quantified with an ultra-sensitive single-molecule (Simoa, Norcross, GA, USA) technology. We explored their consistency and their correlation with clinical severity.Results: Compared with healthy controls, plasma NfL (p < 0.0001) and GFAP (p < 0.001) were increased in Chinese HD mutation carriers, and they were linearly correlated with each other (r = 0.612, p < 0.001). They were also significantly correlated with disease burden, Total Motor Score (TMS) and Total Functional Capacity (TFC). The scores of Stroop word reading, symbol digit modalities tests, and short version of the Problem Behaviors Assessments (PBAs) for HD were correlated with plasma NfL but not GFAP. Compared with healthy controls, plasma NfL has been increased since stage 1 but plasma GFAP began to increase statistically in stage 2.Conclusions: Plasma GFAP was correlated with plasma NfL, disease burden, TMS, and TFC in HD mutation carriers. Plasma GFAP may have potential to be a sensitive biomarker for evaluating HD progression.
format	article
author	Huajing You Tengteng Wu Gang Du Gang Du Yue Huang Yue Huang Yixuan Zeng Lishan Lin Dingbang Chen Chao Wu Xunhua Li Jean-marc Burgunder Zhong Pei
author_facet	Huajing You Tengteng Wu Gang Du Gang Du Yue Huang Yue Huang Yixuan Zeng Lishan Lin Dingbang Chen Chao Wu Xunhua Li Jean-marc Burgunder Zhong Pei
author_sort	Huajing You
title	Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
title_short	Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
title_full	Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
title_fullStr	Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
title_full_unstemmed	Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease
title_sort	evaluation of blood glial fibrillary acidic protein as a potential marker in huntington's disease
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/7597e9b8b9594d0e9cd79cb62e077540
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Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease

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