ArticleThe Journal of biological chemistry2011
Pancreatic lipase-related protein-2 (PLRP2) can contribute to dietary fat digestion in human newborns.
Article in The Journal of biological chemistry, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 40 citations in OpenAlex.
- Characterization of a PNLIP variant identified in Amish pediatric patients with congenital pancreatic lipase deficiency.Journal of lipid research · 2025Article
- Common single-base insertions in the VNTR of the carboxyl ester lipase (CEL) gene are benign and also likely to arise somatically in the exocrine pancreas.Human molecular genetics · 2024Article
- Roles of Milk Fat Globule Membrane on Fat Digestion and Infant Nutrition.Food science of animal resources · 2022Review
- Comparative Study of the Molecular Characterization, Evolution, and Structure Modeling of Digestive Lipase Genes Reveals the Different Evolutionary Selection Between Mammals and Fishes.Frontiers in genetics · 2022Article
- Review
- Structure and Function of Pancreatic Lipase-Related Protein 2 and Its Relationship With Pathological States.Frontiers in genetics · 2021Review
- Single nucleotide polymorphisms in CEL-HYB1 increase risk for chronic pancreatitis through proteotoxic misfolding.Human mutation · 2020Article
- Carboxyl Ester Lipase May Not Mediate Lipotoxic Injury during Severe Acute Pancreatitis.The American journal of pathology · 2019Article
- Influence of PEGylation of Vitamin-K-Loaded Mixed Micelles on the Uptake by and Transport through Caco-2 Cells.Molecular pharmaceutics · 2018Article
- Article
- Genetic risk in chronic pancreatitis: the misfolding-dependent pathway.Current opinion in gastroenterology · 2017Review
- A Carboxyl Ester Lipase (CEL) Mutant Causes Chronic Pancreatitis by Forming Intracellular Aggregates That Activate Apoptosis.The Journal of biological chemistry · 2016Article
- The β5-Loop and Lid Domain Contribute to the Substrate Specificity of Pancreatic Lipase-related Protein 2 (PNLIPRP2).The Journal of biological chemistry · 2015Article
- A novel mutation in PNLIP causes pancreatic triglyceride lipase deficiency through protein misfolding.Biochimica et biophysica acta · 2015Article
- Exploring the relationship between lifestyles, diets and genetic adaptations in humans.BMC genetics · 2015Article
- Porcine pancreatic lipase related protein 2 has high triglyceride lipase activity in the absence of colipase.Biochimica et biophysica acta · 2013Article
- Pancreatic lipase-related protein 2 digests fats in human milk and formula in concert with gastric lipase and carboxyl ester lipase.Pediatric research · 2013Article
- Development of the Digestive System-Experimental Challenges and Approaches of Infant Lipid Digestion.Food digestion · 2012Article
- Bile salt-stimulated lipase plays an unexpected role in arthritis development in rodents.PloS one · 2012Article
- Milk Fat Globules: 2024 Updates.Newborn (Clarksville, Md.)Article
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
In newborn mice, PLRP2 is essential for fat digestion. In human infants, the role of PLRP2 in fat digestion is unclear, as it has poor activity against long-chain triglycerides in vitro. Also, many infants carry a genetic polymorphism resulting in a truncated protein, PLRP2 W340X, which may impact function significantly. We re-examined the properties of recombinant human PLRP2 and studied the impact of W340X mutation on its function. In the presence of bile salt micelles and colipase, human PLRP2 hydrolyzed long-chain tri-, di-, and monoglycerides. It hydrolyzed triolein at a level much lower than that of pancreatic triglyceride lipase, but close to that of carboxyl ester lipase, after a long lag phase, which could be eliminated by the addition of oleic acids. Human PLRP2 W340X was poorly secreted and largely retained inside the cell. The retention of the mutant protein triggered endoplasmic reticulum stress and unfolded protein responses. Our results show that earlier studies underestimated human PLRP2 activity against triolein by employing suboptimal assay conditions. In vivo, dietary fat emulsions contain fatty acids as a result of the action of gastric lipase. Consequently, PLRP2 can contribute to fat digestion during early infancy. Furthermore, infants with homozygous W340X alleles will not secrete functional PLRP2 and may have inefficient dietary fat digestion, particularly when breastfeeding is unavailable. Additionally, the aberrant folding of W340X mutant may cause chronic cellular stress and increase susceptibility of pancreatic exocrine cells to other metabolic stressors.
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