abetalipoproteinemia (ABL) /əbā′təlīp′ōprō′tinē″mē·ə/ [Gk, a + beta, not beta, lipos, fat, proteios, first rank, haima, blood] , a group of rare inherited disorders of fat metabolism, characterized by the absence of apoprotein β-100 and manifested by acanthocytosis, low or absent serum beta-lipoprotein levels, and hypocholesterolemia. In severe cases, steatorrhea, ataxia, nystagmus, motor incoordination, and retinitis pigmentosa occur. Also called Bassen-Kornzweig syndrome.
