Abstract |
High density lipoprotein (HDL) infusion and partial plasma exchange were undertaken in two patients homozygous for Tangier disease. Serum samples and ultracentrifugally isolated serum fractions were analysed over a period of 7 days post infusion by agarose electrophoresis, two-dimensional immunoelectrophoresis (employing antibodies to HDL, HDL3, Apoprotein A-I, and Apoprotein A-II), Apoprotein A radioimmunoassay, and analytical polyacrylamide electrophoresis. The following observations were made: (a) immediately after HDL substitution the broad-beta band, normally visible upon agarose electrophoresis of Tangier plasma, resolved into a distinct beta and pre-beta band; (b)as HDL was catabolized, an abnormal alpha-migrating lipoprotein was generated which contained Apoprotein A-II as protein constituent; and (c) there was a proferential loss of Apoprotein A-I from HDL and the plasma compartment in the course of HDL catabolism. The results suggest that the defect in Tangier disease resides with enhanced catabolism or defective synthesis of Apoprotein A-I.
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Authors | G Assmann, E Smootz |
Journal | European journal of clinical investigation
(Eur J Clin Invest)
Vol. 8
Issue 3
Pg. 131-5
(Jun 1978)
ISSN: 0014-2972 [Print] England |
PMID | 211037
(Publication Type: Journal Article)
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Chemical References |
- Apolipoproteins
- Lipoproteins, HDL
- Cholesterol
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Topics |
- Apolipoproteins
(blood)
- Blood Transfusion
- Cholesterol
(blood)
- Electrophoresis, Agar Gel
- Electrophoresis, Polyacrylamide Gel
- Humans
- Hypolipoproteinemias
(blood)
- Immunoelectrophoresis
- Lipoproteins, HDL
(administration & dosage, blood)
- Tangier Disease
(blood)
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