Rosenberg R N, Banner C
Department of Neurology, University of Texas Southwestern Medical Center, Dallas.
J Neurol Neurosurg Psychiatry. 1989 May;52(5):666-8. doi: 10.1136/jnnp.52.5.666.
Immunochemical analyses (Western blots) of cerebellar homogenates for glutamate dehydrogenase (GDH) from patients with spinocerebellar degeneration and control subjects were conducted. Four patients with autosomal dominant Joseph disease type of spinocerebellar degeneration, one patient with autosomal dominant olivopontocerebellar degeneration and four control subjects were studied. GDH was of the same molecular weight and amount in all patients and control subjects. These data together with normal GDH activity from these same homogenates published previously support the view that GDH is not involved in the pathogenesis of these types of dominantly inherited spinocerebellar degeneration.
对脊髓小脑变性患者和对照受试者的小脑匀浆进行了谷氨酸脱氢酶（GDH）的免疫化学分析（蛋白质印迹法）。研究了4例常染色体显性遗传性脊髓小脑变性约瑟夫病型患者、1例常染色体显性遗传性橄榄体脑桥小脑萎缩患者以及4名对照受试者。所有患者和对照受试者的GDH分子量和含量均相同。这些数据以及之前发表的来自相同匀浆的正常GDH活性数据均支持以下观点：GDH不参与这些类型的显性遗传性脊髓小脑变性的发病机制。
