Electron microscopic structure of serum lipoproteins from patients with fish eye disease
The structure and composition of lipoprotein fractions from two patients with fish eye disease were examined. The composition of very low density lipoproteins (VLDL) was normal, although total mass was greatly elevated. The mean particle sizes of the VLDL were 44.6 +/- 22.2 and 42.8 +/- 19.8 nm for Patients 1 and 2, respectively. Intermediate density lipoprotein (IDL) concentrations in patients were elevated and contained increased triglyceride content; particle sizes for Patients 1 and 2 were 29.4 +/- 3.5 nm and 28.0 +/- 4.1 nm, respectively. In both patients, the triglyceride/cholesteryl ester ratio in LDL was approximately tenfold higher than in normal individuals; however, the LDL particles were somewhat smaller in diameter than those in controls. In both patients, large vesicular structures were occasionally encountered in the LDL region. The high density lipoprotein (HDL) fraction of fish eye disease patients showed the greatest abnormalities. Not only was the total HDL concentration extremely low, but unesterified cholesterol was increased relative to cholesteryl ester. Particle morphology was heterogeneous; the major HDL species was a small spherical particle with a diameter of 7.6 nm. Discoidal particles wtih a thickness of 4.4 nm and diameters between 17.4 and 20.8 nm were also present, together with large (40-90 nm) vesicles. Since lecithin:cholesterol acyltransferase activity levels appear normal is fish eye disease patients, the accumulation of these unusual structures must be due to other metabolic defects such as low total apolipoprotein A-I content and/or association of a polipoprotein E with unesterifed cholesterol-rich particles that inhibit their esterification. 27 references, 4 figures, 2 tables.
- Research Organization:
- Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)
- OSTI ID:
- 6103422
- Journal Information:
- Arteriosclerosis (Dallas); (United States), Vol. 4:2
- Country of Publication:
- United States
- Language:
- English
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Related Subjects
HEREDITARY DISEASES
PATHOLOGY
LIPOPROTEINS
CHEMICAL COMPOSITION
MOLECULAR STRUCTURE
CHOLESTEROL
ELECTRON MICROSCOPY
PARTICLE SIZE
PATIENTS
TRANSFERASES
TRIGLYCERIDES
DISEASES
ENZYMES
ESTERS
HYDROXY COMPOUNDS
LIPIDS
MICROSCOPY
ORGANIC COMPOUNDS
PROTEINS
SIZE
STEROIDS
STEROLS
550900* - Pathology