Literature DB >> 6787335

Uptake of lysosomal enzymes by human fibroblasts: lack of uptake of fungal or plant glycosidases in comparison with a mammalian enzyme.

K C Dooley, D A Applegarth.   

Abstract

alpha-Galactosidase from human placenta was actively taken up by cultured fibroblasts from patients with Fabry's disease whereas the enzyme from the fig was not absorbed. alpha-Glucosidase from two microbiological sources. A. fumigatus and A. niger, was not taken up by cultured fibroblasts from patients with Pompe's disease.

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Year:  1980        PMID: 6787335     DOI: 10.1007/BF02312548

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


  5 in total

1.  Properties of mouse alpha-galactosidase.

Authors:  A J Lusis; K Paigen
Journal:  Biochim Biophys Acta       Date:  1976-07-21

Review 2.  Alpha-glucosidase administration: experiences in two patients with glycogen storage disease compared with animal experiments.

Authors:  F Huijing; B L Waltuck; W J Whelan
Journal:  Birth Defects Orig Artic Ser       Date:  1973-03

3.  Anomeric structure of globoside and ceramide grihexoside of human erythrocytes and hamster fibroblasts.

Authors:  S I Hakomori; B Siddiqui; Y T Li; S C Li; C G Hellerqvist
Journal:  J Biol Chem       Date:  1971-04-10       Impact factor: 5.157

Review 4.  Enzyme replacement in Fabry disease: treatment of cultured skin fibroblasts with a purified alpha-galactosidase from ficin.

Authors:  G Dawson; R Matalon; Y T Li
Journal:  Birth Defects Orig Artic Ser       Date:  1973-03

Review 5.  Treatment related observations in solid tissues, fibroblast cultured and amniotic fluid cells of type II glycogenosis, Hurler disease and metachromatic leukodystrophy.

Authors:  G Hug; W K Schubert; S Soukup
Journal:  Birth Defects Orig Artic Ser       Date:  1973-03
  5 in total
  2 in total

1.  Protein bodies and vacuoles as lysosomes : investigations into the role of mannose-6-phosphate in intracellular transport of glycosidases in pea cotyledons.

Authors:  P R Gaudreault; L Beevers
Journal:  Plant Physiol       Date:  1984-09       Impact factor: 8.340

2.  Loss of electron-dense lamellar material from Fabry's disease fibroblasts after enzyme replacement.

Authors:  R N Sifers; J S Mayes; R E Nordquist
Journal:  Hum Genet       Date:  1983       Impact factor: 4.132

  2 in total

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