abetalipoproteinemia

abetalipoproteinemia or (Brit.) abetalipoproteinaemia an autosomal recessive disorder in which plasma lipoproteins lack apolipoprotein B. There is defective assembly and secretion both of chylomicrons in intestinal mucosa and of very-low-density lipoproteins in the liver. The cause is a deficiency of the 88 kDa subunit of microsomal triglyceride transfer protein.