Lipoproteins containing apolipoprotein B isolated from patients with abetalipoproteinemia and homozygous hypobetalipoproteinemia: identification and characterization.
Abetalipoproteinemia (ABL) and homozygous hypobetalipoproteinemia (HBL) are inherited disorders which are classically characterized by progressive retinal and spinocerebellar disease, fat-soluble vitamin deficiency, and absence of apolipoprotein (apo) B from the plasma. Using immunoaffinity chromatography with an anti-apo B antiserum, we isolated apo B-containing lipoprotein (LpB) particles from the plasma of 4 ABL and 2 HBL patients. The LpB particles were characterized and compared with low density lipoprotein (LDL) and LpB isolated from normal plasma. The ABL/HBL LpB particles were similar in size and charge to normal LpB particles but were relatively enriched in several other apolipoproteins. They contained alpha-tocopherol in a ratio to cholesterol that was proportionately much higher than the very low ratio of alpha-tocopherol to cholesterol in plasma. They bound saturably to fibroblasts and were internalized and degraded similarly to LDL. Hence, the molecular defects in ABL and HBL permit the secretion of a very small number of apo B-containing lipoproteins which may be important for transport of alpha-tocopherol to peripheral tissues.
Aguie
G A
GA
Serlia, Inserm U325, Institut Pasteur, Lille, France.
Rader
D J
DJ
Clavey
V
V
Traber
M G
MG
Torpier
G
G
Kayden
H J
HJ
Fruchart
J C
JC
Brewer
H B
HB
Castro
G
G
D000012:Abetalipoproteinemia / Q000145:classification* / Q000150:complications / Q000235:genetics; D000328:Adult; D053299:Apolipoprotein B-100; D001055:Apolipoproteins B / Q000032:analysis* / Q000097:blood / Q000235:genetics; D002784:Cholesterol / Q000097:blood; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006720:Homozygote; D006801:Humans; D006995:Hypobetalipoproteinemias / Q000097:blood* / Q000150:complications / Q000235:genetics; D008074:Lipoproteins / Q000097:blood / Q000145:classification / Q000302:isolation & purification*; D008077:Lipoproteins, LDL / Q000097:blood; D008297:Male; D008875:Middle Aged; D010316:Particle Size; D014810:Vitamin E / Q000097:blood*; D014811:Vitamin E Deficiency / Q000097:blood / Q000209:etiology
D053299:Apolipoprotein B-100; D001055:Apolipoproteins B; D008074:Lipoproteins; D008077:Lipoproteins, LDL; D014810:Vitamin E; D002784:Cholesterol