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Protracted form of Canavan's disease: case history and protein kinase activity of membrane fractions.

作者信息

Boehme D H, Marks N

出版信息

Acta Neuropathol. 1981;55(3):221-5. doi: 10.1007/BF00691321.

DOI:10.1007/BF00691321
PMID:7349580
Abstract

Clinical and morphological findings in an 8-year-old Jewish girl with spongy leukodystrophy are presented. Ultrastructural changes indicated that this may be a form of Canavan's disease with some atypical features. Biochemical measurements indicated that synaptosomal membranes prepared from the striatum but not from the cerebral cortex were deficient in protein kinase activity based on incorporation of 32P into proteins. Both areas contained the same spectrum of proteins. Cyclic AMP (cAMP) measurements indicated no significant differences between brain areas, or when compared to tissue obtained from a stillborn and a 5-year-old child.

摘要

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1
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引用本文的文献

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Canavan disease. Analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms.卡纳万病。对导致所观察到的临床症状发展的代谢性病变性质的分析。
J Mol Neurosci. 1997 Oct;9(2):109-25. doi: 10.1007/BF02736855.

本文引用的文献

1
Protracted form of spongy degeneration of the central nervous system (van Bogaert and Bertrand type).
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2
Spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan's disease). A review.
Hum Pathol. 1973 Sep;4(3):331-47. doi: 10.1016/s0046-8177(73)80098-x.
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Protein synthesis by synaptosomes from rat brain. Contribution by the intraterminal mitochondria.大鼠脑突触体的蛋白质合成。终末内线粒体的作用。
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6
[Hereditary spongiform dystrophy in young children (Canavan: van Bogaert-Bertrand)].幼儿遗传性海绵状营养不良(卡纳万病:范博加特 - 贝特朗病)
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7
[Juvenile spongy dystrophy of CNS with necrosis of the medulla. A. complication of hydroxyquinoline therapy (author's transl)].[伴有延髓坏死的中枢神经系统青少年海绵状营养不良。A. 羟基喹啉治疗的并发症(作者译)]
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Spongy degeneration of the central nervous system (van Bogaert-Bertrand type?) in a newborn infant. A light and electron microscopic study.
Acta Neuropathol. 1977 Mar 31;37(3):267-70. doi: 10.1007/BF00686890.
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Congenital spongy degeneration of the brain (van Bogaert - Bertrand) associated with micrencephaly and ponto - cerebellar atrophy (contributions to the pathology of glial dystrophy of intrauterin origin).
Neuropadiatrie. 1977 Feb;8(1):73-87. doi: 10.1055/s-0028-1091507.
10
[Spongious cerebral dystrophy at an infant age (Canavan-Bogaert-Bertrand types) in three siblings of a non-Jewish family in upper Franconia (author's transl)].
Klin Padiatr. 1978 Nov;190(6):580-5.