Bassen-Kornzweig syndrome consists of Abetalipoproteinemia,Acanthocytosis and Apolipoprotein B deficiency
Bassen-Kornzweig syndrome is a rare, autosomal recessive disorder in which the microsomal triglyceride transfer protein (MTP) is absent because of mutations in the MTP gene. Individuals who are homozygous for this disorder have total cholesterol levels <1.3 mmol/L (<50 mg/dL) and essentially no VLDL, IDL, LDL, or chylomicrons.
Bassen-Kornzweig syndrome is a rare disease passed down through families more often affecting males,in which a person is unable to fully absorb dietary fats through the intestines.
Because dietary fat as well as vitamins A and E are transported from the intestine in chylomicrons, these patients may have malabsorption of fat and fat-soluble vitamins. Vitamin E deficiency in infancy and early childhood can result in neurologic problems. Moderately low levels of total cholesterol may also be associated with extreme reductions in HDL cholesterol; as noted above, these are almost always secondary to mutations in the gene for apo AI and a lack of apo AI in plasma.