Literature DB >> 3123795

Metabolism of cerebroside sulphate and subcellular distribution of its metabolites in cultured skin fibroblasts derived from controls, metachromatic leukodystrophy, globoid cell leukodystrophy and Farber disease.

K Inui1, M Furukawa, J Nishimoto, S Okada, H Yabuuchi.   

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Year:  1987        PMID： 3123795     DOI： 10.1007/bf01800085

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  8 in total

1.  Cerebroside sulfate hydrolysis by fibroblasts from a parent with metachromatic leukodystrophy.

Authors:  A L Fluharty; R L Stevens; H Kihara
Journal:  J Pediatr       Date:  1978-05       Impact factor: 4.406

2.  Fluorescence polarization analysis, lipid composition, and Na+, K+-ATPase kinetics of synaptosomal membranes in feline GM1 and GM2 gangliosidosis.

Authors:  P A Wood; M R McBride; H J Baker; S T Christian
Journal:  J Neurochem       Date:  1985-03       Impact factor: 5.372

3.  Hexosaminidase A activity in skin fibroblasts from various types of GM2 gangliosidosis using a fluorogenic sulphated substrate.

Authors:  K Inui; T Yutaka; S Okada; H Yabuuchi; D A Wenger; R J Desnick
Journal:  J Inherit Metab Dis       Date:  1985       Impact factor: 4.982

4.  Stearoyl[1-14C]sulfogalactosylsphingosine ([14C]sulfatide) as substrate for cerebroside sulfatase assay.

Authors:  G Dubois; B Zalc; F Le Saux; N Baumann
Journal:  Anal Biochem       Date:  1980-03-01       Impact factor: 3.365

5.  Distortion of neuronal geometry and formation of aberrant synapses in neuronal storage disease.

Authors:  D P Purpura; K Suzuki
Journal:  Brain Res       Date:  1976-10-29       Impact factor: 3.252

6.  Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein.

Authors:  K Inui; E E Grebner; L G Jackson; D A Wenger
Journal:  Am J Hum Genet       Date:  1983-07       Impact factor: 11.025

7.  Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts.

Authors:  T Kudoh; D A Wenger
Journal:  J Clin Invest       Date:  1982-07       Impact factor: 14.808

8.  Intracellular translocation of fluorescent sphingolipids in cultured fibroblasts: endogenously synthesized sphingomyelin and glucocerebroside analogues pass through the Golgi apparatus en route to the plasma membrane.

Authors:  N G Lipsky; R E Pagano
Journal:  J Cell Biol       Date:  1985-01       Impact factor: 10.539
  8 in total
  3 in total

1.  A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease.

Authors:  Carmen Bedia; Luz Camacho; José Luís Abad; Gemma Fabriàs; Thierry Levade
Journal:  J Lipid Res       Date:  2010-09-24       Impact factor: 5.922

2.  Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease.

Authors:  J Tohyama; Y Oya; T Ezoe; M T Vanier; H Nakayasu; N Fujita; K Suzuki
Journal:  J Inherit Metab Dis       Date:  1999-06       Impact factor: 4.982

3.  Functional compartments of sulphatide metabolism in cultured living cells: evidence for the involvement of a novel sulphatide-degrading pathway.

Authors:  M C Tempesta; R Salvayre; T Levade
Journal:  Biochem J       Date:  1994-02-01       Impact factor: 3.857
  3 in total

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