Abstract
During the last two decades, a large body of information on the events responsible for intestinal fat digestion and absorption has been accumulated. In particular, many groups have extensively focused on the absorptive phase in order to highlight the critical “players” and the main mechanisms orchestrating the assembly and secretion of chylomicrons (CM) as essential vehicles of alimentary lipids. The major aim of this article is to review understanding derived from basic science and clinical conditions associated with impaired packaging and export of CM. We have particularly insisted on inborn metabolic pathways in humans as well as on genetically modified animal models (recapitulating pathological features). The ultimate goal of this approach is that “experiments of nature” and in vivo model strategy collectively allow gaining novel mechanistic insight and filling the gap between the underlying genetic defect and the apparent clinical phenotype. Thus, uncovering the cause of disease contributes not only to understanding normal physiologic pathway, but also to capturing disorder onset, progression, treatment and prognosis.
Highlights
Intestinal fat transport is a prerequisite process to deliver alimentary lipids to the bloodstream for subsequent metabolism and peripheral energy homeostasis
The objective of the present review is to focus on congenital disorders of intestinal lipid absorption, highlighting the molecular genetics and pathophysiological mechanisms while describing the clinical manifestations and management of patients
L-FABP is abundantly detected in the small intestine and has broad fatty acid (FA) binding specificity with high affinity for long-chain polyunsaturated FAs though it may bind to CHOL, acyl-CoA, bile acid, and phytanic acid (Gordon et al, 1985; Lowe et al, 1987; Thumser and Wilton, 1996; Wolfrum et al, 1999)
Summary
Intestinal fat transport is a prerequisite process to deliver alimentary lipids to the bloodstream for subsequent metabolism and peripheral energy homeostasis. Physiological and morphological requirements must be met to allow intraluminal digestion and intracellular transport of triglycerides (TG), phospholipids (PL), and cholesterol (CHOL). An Update on Intestinal Fat Regulation/Absorption and mucosal transport represent the essential steps to warrant optimal lipid absorption. Their abnormalities result in intestinal fat malabsorption of lipids, and of fat-soluble vitamins, concomitantly with gastrointestinal (GI) symptoms along with steatorrhea, nutritional fatty acid (FA) and vitamin deficiency, and substantial extra-intestinal disorders. The objective of the present review is to focus on congenital disorders of intestinal lipid absorption, highlighting the molecular genetics and pathophysiological mechanisms while describing the clinical manifestations and management of patients
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