Abstract
Intestinal lipid transport is a multistep process encompassing pathways that facilitate incorporation of fatty acids, monoglycerides, lipovitamins, and sterol substrates into a complex lipid for systemic delivery. These steps include transport across the brush border membrane and vectorial delivery within apical cytoplasmic compartments to the endoplasmic reticulum (ER) where complex lipid is synthesized and initiation of lipoprotein biogenesis occurs. Within the ER, primordial particles are remodeled and undergo maturation through the Golgi profiles, and nascent intracellular chylomicrons are eventually secreted into the pericellular basolateral spaces and lymphatic fenestrae. We review the current understanding of intestinal lipoprotein biogenesis and the role of dominant genes in coordinating chylomicron assembly and secretion. We further discuss developments in the mechanisms whereby intestinal lipid (including fatty acid and sterol) transport is disrupted in selected genetic disorders — a topic that provides insight into the physiological function of critical pathways and their physiologic regulation and potential utility as pharmacologic targets.
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