Linked Life Data

6725246

Source:http://linkedlifedata.com/resource/pubmed/id/6725246

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
1984-7-2
pubmed:abstractText
Tangier disease is a disorder characterized by low levels of apo-A-I and high density lipoproteins. The defect in Tangier disease is an abnormal A-I apolipo protein, designated apo-A- ITangier . In normal subjects, apo-A-I is secreted as proapo -A-I with subsequent extracellular conversion to mature apo-A-I. The major form in normal plasma is mature apo-A-I with small amounts of proapo -A-I. In Tangier disease, proapo -A- ITangier is present in roughly equivalent concentrations compared to mature apo-A- ITangier . It has been proposed that the defect in Tangier disease is in the conversion of pro- to mature apo-A- ITangier . To test this, proapo -A-I was isolated from normal and Tangier subjects, and the conversion to the mature form by plasma from normal and Tangier subjects was analyzed. Incubation of radiolabeled normal proapo -A-I in normal plasma anticoagulated with heparin was associated with progressive conversion to mature apo-A-I over 24 h (initially 85% of the radioactivity was in the proapo -A-I isoform; at 24 h 33% radioactivity remained in the pro-isoform). Proapo -A- ITangier was also converted to the mature isoform during 24 h of incubation in normal plasma. Initially, 84% of radioactivity was in proapo -A- ITangier , and by 24 h the radioactivity in this isoprotein had decreased to 36%. A similar pattern of conversion was also observed when proapo -A- ITangier was incubated in Tangier plasma. The proteolytic conversion of both normal proapo -A-I and proapo -A- ITangier was unaffected by the serine protease inhibitors phenylmethylsulfonyl fluoride (1 mM) or aprotinin (200 Kallikrein-inactivating units/ml), but was inhibited by EDTA (0.1%). These results indicate that proapo -A- ITangier can be converted to mature apo-A- ITangier by the converting enzyme in normal plasma. In addition, plasma from a Tangier subject can convert both normal and Tangier proapo -A-I to the mature form. These results establish that proapo -A- ITangier can be rapidly converted to mature apo-A- ITangier , and there is no deficiency of the converting enzyme activity in Tangier disease.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0021-9258
pubmed:author
pubmed:issnType
Print
pubmed:day
25
pubmed:volume
259
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
6049-51
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1984
pubmed:articleTitle
Tangier disease. In vitro conversion of proapo-A-ITangier to mature APO-A-ITangier.
pubmed:publicationType
Journal Article