Literature DB >> 7349580

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

D H Boehme, N Marks.   

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.

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Year:  1981        PMID: 7349580     DOI: 10.1007/bf00691321

Source DB:  PubMed          Journal:  Acta Neuropathol        ISSN: 0001-6322            Impact factor:   17.088


  12 in total

1.  [Hereditary spongiform dystrophy in young children (Canavan: van Bogaert-Bertrand)].

Authors:  P Mirimanoff
Journal:  J Neurol Sci       Date:  1976-06       Impact factor: 3.181

2.  Regulation of endogenous phosphorylation of specific proteins in synaptic membrane fractions from rat brain by adenosine 3':5'-monophosphate.

Authors:  T Ueda; H Maeno; P Greengard
Journal:  J Biol Chem       Date:  1973-12-10       Impact factor: 5.157

3.  Synaptic plasma membranes from rat brain synaptosomes: isolation and partial characterization.

Authors:  C W Cotman; D A Matthews
Journal:  Biochim Biophys Acta       Date:  1971-12-03

4.  Protracted form of spongy degeneration of the central nervous system (van Bogaert and Bertrand type).

Authors:  M Adachi; B W Volk
Journal:  Neurology       Date:  1968-11       Impact factor: 9.910

5.  Spongy degeneration of the central nervous system (van Bogaert-Bertrand type?) in a newborn infant. A light and electron microscopic study.

Authors:  J Towfighi; Z Friedman; M J Maisels
Journal:  Acta Neuropathol       Date:  1977-03-31       Impact factor: 17.088

6.  Basal, cAMP- and cGMP-dependent protein kinases in human brain tumors.

Authors:  L Frattola; C Tonini; R Villani; M Trabucchi
Journal:  Acta Neurol Scand       Date:  1979-12       Impact factor: 3.209

7.  [Juvenile spongy dystrophy of CNS with necrosis of the medulla. A. complication of hydroxyquinoline therapy (author's transl)].

Authors:  J C Azubuike; F Gullotta; H C Kallfelz; K Gellissen; S Mende; R Exss
Journal:  Neuropadiatrie       Date:  1975-08

8.  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).

Authors:  O Vuia
Journal:  Neuropadiatrie       Date:  1977-02

9.  [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)].

Authors:  H Schmidt; H D Rott; G Neuhäuser; W Neumann
Journal:  Klin Padiatr       Date:  1978-11       Impact factor: 1.349

Review 10.  Spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan's disease). A review.

Authors:  M Adachi; L Schneck; J Cara; B W Volk
Journal:  Hum Pathol       Date:  1973-09       Impact factor: 3.466

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  1 in total

Review 1.  Canavan disease. Analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms.

Authors:  M H Baslow; T R Resnik
Journal:  J Mol Neurosci       Date:  1997-10       Impact factor: 3.444

  1 in total

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