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Journal of Lipid Research, Vol 32, 1539-1570, Copyright © 1991 by Lipid Research, Inc.
Structure and function of lamellar bodies, lipid-protein complexes involved in storage and secretion of cellular lipids
G Schmitz and G Muller
Institut fur Klinische Chemie und Laboratoriumsmedizin, Universitat Regensburg, Germany.
This review article attempts to present an overview of the occurrence and
function of lipid storage and secretory organelles: the lamellar bodies.
Morphologically these organelles vary considerably in size (100 nm to 2400
nm); they are surrounded by a membrane and contain multilamellar lipid
membranes. Lamellar bodies may also contain apolipoproteins and lytic
enzymes and have an acidic pH, which confers on them a lysosomal character.
Under normal physiological conditions, the main function of lamellar bodies
is the supply of extracellular domains with specialized lipid components
related to a specialized function. The lamellar bodies of the lung
epithelium are best investigated in their functional and structural
features and are the storage form of the lung surfactant. They provide a
monomolecular lipid film of dipalmitoyl phosphatidylcholine (DPPC) on the
surface of lung alveoli to lower surface tension necessary for optimal gas
exchange and a hydrophobic protective lining against environmental
influences. Additional cells of the respiratory system such as the mucosa
of the human nose and the bronchi contain lamellar bodies. Lamellar bodies
are also found in the gastrointestinal tract, in tongue papillae, oral
epithelium, and mucosa cells of the stomach. The major phospholipid of
lamellar bodies in mucosa cells of the stomach is DPPC, providing a
hydrophobic protective lipid film against the tissue-damaging activities of
gastric juice. The hydrophobic water-protective barrier of the skin, which
consists mainly of neutral lipids, however, also originates from lamellar
bodies secreted by epithelial cells. Lamellar bodies, mainly consisting of
DPPC, also occur in mesodermal cell layers of sliding surfaces to provide
the lubrication of joints, of the peritoneum, pericardium, and pleural
mesothelium. In certain pathological conditions, such as atherosclerosis,
Niemann-Pick disease, lecithin:cholesterol acyltransferase (LCAT)
deficiency, cholestasis, degeneration of nerves and brain, and regeneration
of nerves and wound healing, lipid-containing lamellar bodies have been
observed in various cells, the function of which still remains to be
elucidated. In early and late lesions of atherosclerotic plaques, lamellar
bodies, consisting of unesterified cholesterol and phospholipids, are
associated with the extracellular matrix of the intima. During regression
of fatty streaks, lamellar bodies are seen intracellularly in macrophages
and smooth muscle cells. Inherited metabolic disorders, such as
Niemann-Pick disease type I and type II, result in the excessive
accumulation of lamellar body-containing cells, for example in bone marrow,
spleen, and lymphoid tissue. Type I is a deficiency in sphingomyelinase and
type II is a defect in intracellular trafficking of lipoprotein-derived
cholesterol.(ABSTRACT TRUNCATED AT 400 WORDS)

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Copyright © 1991 by the American Society for Biochemistry and Molecular Biology.
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