The lipoprotein abnormality in Tangier disease: quantitation of A apoproteins.

Abstract

In this study we have determined by radioimmunoassay and double immunoelectrophoresis the total quantities and distributions of A apoproteins in three adult patients affected with Tangier disease (hereditary alpha-lipoprotein deficiency). Compared with normal plasma, the total quantities of apoproteins A-I and A-II in Tangier plasma were determined to be less than 1% and 5-7%, respectively. In Tangier patients, approximately 90% of the apoprotein A-I sedimented when ultracentrifugations of plasma were carried out at density 1.21 g/ml KBr. By contrast, more than 95% of the apoprotein A-II floated under those conditions. In normal plasma, approximately 90% of both apoproteins A-I and A-II is found in the 1.063-1.21-g/ml KBr density fraction. These findings suggest that complete dissociation of A apoproteins occurs in Tangier plasma. This dissociation of apoproteins was confirmed by double immunoelectrophoresis with monospecific antisera. Immunochemical and electrophoretic experiments did not provide evidence for a structural abnormality of apoprotein A-I in these patients, The results taken together strongly suggest that normal high-density lipoproteins are absent from Tangier plasma.

• Authors: G Assmann, E Smootz, K Adler, A Capurso, K Oette
• Journal: The Journal of clinical investigation (J Clin Invest) Vol. 59 Issue 3 Pg. 565-75 (Mar 1977) ISSN: 0021-9738 [Print] United States
• PMID: 190272 (Publication Type: Journal Article)

Chemical References

• Apolipoproteins
• Apoproteins
• Lipoproteins, HDL

Topics

• Adult
• Apolipoproteins (blood)
• Apoproteins (blood)
• Blood Protein Disorders (blood, genetics)
• Female
• Humans
• Immunoelectrophoresis
• Lipoproteins, HDL (deficiency)
• Male
• Middle Aged
• Radioimmunoassay