Linked Life Data

8902191

Source:http://linkedlifedata.com/resource/pubmed/id/8902191

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1997-2-28
pubmed:abstractText
Mucolipidosis type 4 (ML-4) is an autosomal recessive inborn error of metabolism, the pathogenesis of which is not known. We characterized protein kinase C (PKC) activation and cellular phosphate uptake in intact quiescent ML-4 skin fibroblasts and after stimulation with phorbol myristate acetate (PMA). [3H]Phorbol dibutyrate uptake was not altered in ML-4 compared to control cells. Translocation of PKC from the cytosolic to the membranous compartment upon stimulation with PMA was perturbed in ML-4 cells. Phosphate uptake was reduced in both cytosolic and membranous fractions of quiescent ML-4 cells. Stimulation with PMA did not elicit an increase in phosphate uptake in the cytosolic fraction of ML-4 cells compared with control cells, but led to comparable phosphate uptake in the membranous fraction of both cell types. The data indicate that PKC-mediated signal transduction may be perturbed in ML-4. Other kinases and phosphatases may be involved. These alterations may play an important role in the pathogenesis of this disorder.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1077-3150
pubmed:author
pubmed:issnType
Print
pubmed:volume
59
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
33-7
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1996
pubmed:articleTitle
Protein kinase C activation and phosphate uptake are altered in intact mucolipidosis type-4 skin fibroblasts.
pubmed:affiliation
Department of Paediatrics, Hadassah University Hospital, Mt. Scopus, Jerusalem, Israel.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't