Familial apolipoprotein Al and apolipoprotein CIII deficiency: subclass distribution, composition, and morphology of lipoproteins in a disorder associated with premature atherosclerosis

Journal Article · Thu Nov 01 00:00:00 EST 1984 · J. Clin. Invest.; (United States)

DOI:https://doi.org/10.1172/JCI111576· OSTI ID:5244266

Forte, T M; Nichols, A V; Krauss, R M; Norum, R A

Lipoprotein classes isolated from the plasma of two patients with apolipoprotein AI (apo AI) and apolipoprotein CIII (apo CIII) deficiency were characterized and compared with those of healthy, age- and sex-matched controls. The plasma triglyceride values for patients 1 and 2 were 31 and 51 mg/dl, respectively, and their cholesterol values were 130 and 122 mg/dl, respectively; the patients, however, had no measurable high density lipoprotein (HDL)-cholesterol. Analytic ultracentrifugation showed that patients' S/sub f//sup 0/ 0-20 lipoproteins posses a single peak with S/sub f//sup 0/ rates of 7.4 and 7.6 for patients 1 and 2, respectively, which is similar to that of the controls. The concentration of low density lipoprotein (LDL) (S/sub f//sup 0/ 0-12) particles, although within normal range (331 and 343 mg/dl for patients 1 and 2, respectively), was 35% greater than that of controls. Intermediate density lipoproteins (IDL) and very low density lipoproteins (VLDL) (S/sub f//sup 0/ 20-400) were extremely low in the patients. HDL in the patients had a calculated mass of 15.4 and 11.8 mg/dl for patients 1 and 2, respectively. No HDL could be detected by analytic ultracentrifugation, but polyacrylamide gradient gel electrophoresis (gge) revealed that patients possessed two major HDL subclasses: (HDL/sub sb/)/sub gge/ at 11.0 nm and (HDL/sub 3b/)/sub gge/ at 7.8 nm. The major peak in the controls (HDL/sub 3a/)/sub gge/, was lacking in the patients.

• Research Organization: Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)
• OSTI ID: 5244266
• Journal Information: J. Clin. Invest.; (United States), Vol. 74
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
ARTERIOSCLEROSIS
PATHOGENESIS
CHOLESTEROL
BLOOD CHEMISTRY
HEMIC DISEASES
HEREDITARY DISEASES
LIPOPROTEINS
TRIGLYCERIDES
BLOOD PLASMA
ELECTROPHORESIS
PATIENTS
BIOLOGICAL MATERIALS
BLOOD
BODY FLUIDS
CARDIOVASCULAR DISEASES
DISEASES
ESTERS
HYDROXY COMPOUNDS
LIPIDS
MATERIALS
ORGANIC COMPOUNDS
PROTEINS
STEROIDS
STEROLS
VASCULAR DISEASES
550900* - Pathology
550200 - Biochemistry