Linked Life Data

409577

Source:http://linkedlifedata.com/resource/pubmed/id/409577

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1977-11-30
pubmed:abstractText
The release of acid hydrolases from cultured skin fibroblasts into the cell culture medium was studied in several lysosomal storage disorders (GM1-gangliosidosis, Fabry's disease, Hurler's disease, mannosidosis, and mucolipidosis). The levels of different activities were proportional to time (up to 44 h after medium change) and cell density with the exception of beta-glucosidase, which was not released. Culture medium from the fibroblasts of mucolipidosis patients exhibited higher activity of acid hydrolases than medium from cells of patients with GM1-gangliosidosis, Fabry's disease, Hurler's disease, and mannosidosis. These cells, however, exhibited somewhat higher levels of enzyme activity in their culture medium than control fibroblasts. The total production of acid hydrolases was yet rather similar in fibroblasts from controls and patients. Differential centrifugation showed that the highest specific activity of acid hydrolases was seen, as expected, in the lysosomal fraction, except in fibroblasts from patients with mucolipidosis, where the supernatant exhibited most activity. beta-Glucosidase, however, showed a normal differential centrifugation pattern also in fibroblasts from these patients.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0009-8981
pubmed:author
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
80
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
79-86
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1977
pubmed:articleTitle
Lysosomal enzymes in medium from cultured skin fibroblasts from normal individuals and patients with lysosomal diseases.
pubmed:publicationType
Journal Article, In Vitro