Sensory and Sympathetic Denervation in the Foot Pads of Mice with a Defect in Bullous Pemphigoid Antigen 1 (BPAG1) Gene

BPAG1基因缺損小鼠的足墊皮膚內感覺神經與交感神經退化之研究

李弘裕(Hung-Yu Li) ； 林培正(David P-C Lin) ； 鄭靜瑩(Chin-Yin Chen) ； 廖克剛(Ko-Kaung Liao) ； 劉春蘭(Chun-Lan Liu) ； 彭美玲(Mei-Lin Peng) ； 溫揚正(Yang-Cheng Wen) ； 曾廣文(Kuang-Wen Tseng)

《中山醫學雜誌》 21卷2期 (2010/06) Pp. 189-199

https://doi.org/10.30096/CSMJ.201006.0006

摘要

Dystonia musculorum(dt)小鼠是自體隱性遺傳bullous pemphigoid antigen1(BPAG1)基因缺損的突變鼠。於正常狀態，包括中樞神經系統與週邊神經系統內，許多神經組織均會大量表達神經型BPAG1基因，但之前基因缺損dt小鼠的研究主要都專注於感覺神經元細胞的退化，而神經型BPAG1基因的缺損對於交感神經的神經支配造成影響的研究則相對付之闕如。為了印證dt小鼠週邊神經的退化情況，所以本實驗分別利用接合廣泛性神經標誌蛋白protein gene product 9.5(PGP 9.5)和交感神經元標誌蛋白酪胺酸羥化叠(tyrosine hydroxylase, TH)的抗體，以皮膚切片進行免疫組織化學染色，從形態觀察神經支配的情形，並與正常小鼠做比較。實驗證實，dt小鼠腳掌足墊處皮膚在接合PGP 9.5與TH這兩種抗體標定的免疫染色與正常小鼠相較都有顯著差異，根據以上結果，我們認為細胞骨架接合蛋白BPAG1基因缺損的dt小鼠，不但感覺神經的退化，皮膚汗腺處的交感神經亦明顯有神經退化的現象。

關鍵字

皮膚表面去神經化 ； 神經退化 ； 細胞骨架蛋白 ； 交感神經節 ； dt基因突變

並列摘要

Dystonia musculorum (dt) mice are autosomal recessive hereditary mutants with a defect in the bullous pemphigoid antigen 1 (BPAG1) gene. The neural isoform of BPAG1 (BPAG1) is expressed in various neurons, including those in the central and peripheral nervous systems of wild-type mice. Most previous studies on BPAG1- deficient mice have focused on sensory neuronal degeneration; limited investigation of sympathetic innervations has been conducted. To investigate the degeneration of peripheral nerves, patterns of nerve innervation in the skin of dt mice were observed and compared with those in wild-type mice. Nerve innervation was assayed using a general neuronal marker protein gene product 9.5 (PGP 9.5) and the sympathetic neuronal marker tyrosine hydroxylase (TH) with immunoreactivity. Quantitative results indicate that the deficiency in the cytoskeletal linker BPAG1 is responsible for the significant down-regulation in peripheral sensory nerves and sympathetic innervation of the sweat gland in the skin of dt mice foot pads.

並列關鍵字

cutaneous denervation ； nerve degeneration ； cytoskeleton ； sympathetic ganglia ； dt mutant