Lipoprotein lipase with a defect in lipid interface recognition in a case with type I hyperlipidaemia

European Journal of Clinical Investigation
J KobayashiS Yoshida

Abstract

Defective lipoprotein lipase (LpL) was found in the postheparin plasma (PHP) of a patient with severe hypertriglyceridaemia. The patient was a 14-year-old girl with a maximum plasma triglyceride (TG) level of 3600 mg d-1 who had been suffering from recurrent pancreatitis. The patient's LpL purified from the PHP by heparin-Sepharose and phenyl-Sepharose chromatographies hydrolysed tributryrin, but not triolein emulsified with Triton X-100 and phosphatidylcholine (PC), or in chylomicrons, whereas normal LpL hydrolysed these substrates. Moreover, unlike normal LpL, LpL from the patient did not associate with VLDL, as shown by Sepharose 4B column chromatography. The patient's LpL hydrolysed triolein emulsified with lysophospholipid at a normal rate in the presence of apolipoprotein CII. These findings suggest that this patient has LpL with a normal catalytic site for tributyrin but with a defect in lipid interface recognition resulting in loss of ability to recognize VLDL or chylomicrons, but not of triolein emulsified with lysophospholipid.

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Citations

Jul 16, 1993·Clinica Chimica Acta; International Journal of Clinical Chemistry·J KobayashiS Yoshida
Oct 6, 2000·Clinica Chimica Acta; International Journal of Clinical Chemistry·S HirayamaY Saito
Aug 28, 1998·Clinica Chimica Acta; International Journal of Clinical Chemistry·T MuranoK Shirai
Sep 11, 1999·Clinica Chimica Acta; International Journal of Clinical Chemistry·J KobayashiY Saito
Mar 4, 2000·Clinica Chimica Acta; International Journal of Clinical Chemistry·J KobayashiY Saito
Jan 22, 2010·Artificial Organs·Claudia StefanuttiPiero Marson
Jan 29, 2011·Journal of Lipid Research·Debapriya BasuWeijun Jin
Feb 24, 2001·Clinical Chemistry and Laboratory Medicine : CCLM·J LiT Kanno
Oct 29, 2003·Clinical Chemistry and Laboratory Medicine : CCLM·Jiangzhen LiMasato Maekawa
Dec 1, 1992·Scandinavian Journal of Clinical and Laboratory Investigation·H InaderaS Yoshida
Nov 9, 2006·Journal of Lipid Research·S ImamuraH Mabuchi
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Jan 19, 1996·Biochimica Et Biophysica Acta·P CarreroM A Lasunción

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