Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Overview of Lipid Metabolism

Overview of Lipid Metabolism

Lipid metabolism is a crucial process in the human body that involves the synthesis and degradation of lipids. This process is essential for energy production, cell membrane formation, and hormone production, among other functions.
Lipolysis: The Breakdown of Lipids:
Lipolysis is the process of breaking down lipids, particularly triglycerides, into glycerol and fatty acids. This process typically occurs in the adipose tissue and is triggered by various hormones, including glucagon and...

Lysosomal Hydrolases

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...

Blood Studies for Cardiovascular System III: Serum Lipid Profile

Understanding serum lipids is crucial for maintaining cardiovascular health and preventing heart disease and stroke.
Serum lipids are fats and fatty substances in the blood and are crucial for various bodily functions, including energy storage, cellular structure, and hormone production. Serum lipids consist of cholesterol, triglycerides, and phospholipids.
Cholesterol is a soft, fat-like substance found in all body cells. It is crucial for producing hormones, vitamin D, and substances that aid...

Lipid Catabolism

Lipid Catabolism

Triglycerides serve as crucial long-term energy storage molecules in microorganisms, providing a dense source of metabolic energy. Their breakdown is mediated by lipases, which hydrolyze triglycerides into glycerol and free fatty acids. Each of these components follows distinct metabolic pathways, ultimately contributing to ATP synthesis and cellular energy homeostasis.Glycerol MetabolismGlycerol, released from triglyceride hydrolysis, is phosphorylated by glycerol kinase to form...

Lipid Digestion

Lipid Digestion

Lipids are large molecules that are generally not water-soluble. Since most of the digestive enzymes in the human body are water-based, there are specific steps the body must take to break down lipids and make them available for use.

Lipid Absorption

Lipid Absorption

Dietary triglycerides from chyme in the duodenum are mixed with bile salts produced by the liver to emulsify fats. As a result, large droplets are broken down into smaller ones, increasing the surface area for enzymatic action. Once emulsified, pancreatic lipases hydrolyze the triglycerides into free fatty acids and monoglycerides.
These breakdown products bind with bile salts and lecithin to form micelles, which quickly pass between microvilli to come in close contact with the apical...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Impact of Enzyme Replacement Therapy on Patients with Late Onset Pompe Disease - Real World Data from a Developing Country.

Indian journal of pediatrics·2026

Same author

Clinical, biochemical and molecular spectrum of acute neuronopathic type 2 Gaucher disease from India.

BMC pediatrics·2026

Same author

Systematic analysis of homozygous autosomal copy number losses in exomes improves diagnostic yield and uncovers ultra-rare recessive disorders.

European journal of human genetics : EJHG·2026

Same author

Long-term visual outcomes and treatment-related prognostic variables in pediatric demyelinating optic neuritis: A 10-year retrospective cohort study.

Multiple sclerosis and related disorders·2026

Same author

Wall shear stress and oscillatory shear index are independently associated with complicated carotid artery plaques.

European heart journal. Cardiovascular Imaging·2025

Same author

Cardiomyocyte-Specific Deletion of Sirtuin 5 Accelerates the Development of Heart Failure Upon Dysregulating Purine Metabolism.

Acta physiologica (Oxford, England)·2025

Search research articles

Related Experiment Video

Updated: Dec 20, 2025

Author Spotlight: Analysis of Fluorescent-Stained Lipid Droplets with 3D Reconstruction for Hepatic Steatosis Assessment

Author Spotlight: Analysis of Fluorescent-Stained Lipid Droplets with 3D Reconstruction for Hepatic Steatosis Assessment

Published on: June 2, 2023

Lipoprotein Lipase Deficiency.

Shwetha Kuthiroly¹, Dhanya Yesodharan¹, Natasha Radhakrishnan²

¹Department of Pediatric Genetics, Amrita Institute of Medical Sciences & Research Centre, Aims Ponekkara PO, Cochin, Kerala, 682041, India.

Indian Journal of Pediatrics

|May 31, 2020

Summary

This summary is machine-generated.

Lipoprotein Lipase (LPL) deficiency in children presents with severe hypertriglyceridemia. A fat-restricted diet significantly improves lipid profiles and prevents pancreatitis, aiding in LPL deficiency management.

Keywords:

Diet Lipemia retinalis Lipemic plasma Lipoprotein lipase deficiency Pancreatitis

More Related Videos

Isolation and Analysis of Plasma Lipoproteins by Ultracentrifugation

Isolation and Analysis of Plasma Lipoproteins by Ultracentrifugation

Published on: January 28, 2021

Exploring the Regulation of Lipid Droplet Catabolism through Lipophagy

Exploring the Regulation of Lipid Droplet Catabolism through Lipophagy

Published on: January 31, 2025

Related Experiment Videos

Last Updated: Dec 20, 2025

Author Spotlight: Analysis of Fluorescent-Stained Lipid Droplets with 3D Reconstruction for Hepatic Steatosis Assessment

Author Spotlight: Analysis of Fluorescent-Stained Lipid Droplets with 3D Reconstruction for Hepatic Steatosis Assessment

Published on: June 2, 2023

Isolation and Analysis of Plasma Lipoproteins by Ultracentrifugation

Isolation and Analysis of Plasma Lipoproteins by Ultracentrifugation

Published on: January 28, 2021

Exploring the Regulation of Lipid Droplet Catabolism through Lipophagy

Exploring the Regulation of Lipid Droplet Catabolism through Lipophagy

Published on: January 31, 2025

Area of Science:

Biochemistry
Genetics
Pediatrics

Background:

Lipoprotein Lipase (LPL) deficiency is a rare genetic disorder characterized by severe hypertriglyceridemia.
Clinical manifestations include hepatosplenomegaly, lipemia retinalis, and potential for pancreatitis and chylothorax.
Early diagnosis and management are crucial to prevent severe complications.

Purpose of the Study:

To analyze the clinical and molecular spectrum of LPL deficiency.
To evaluate the effectiveness of a cost-effective indigenous diet in managing LPL deficiency.

Main Methods:

A single-center retrospective study of 15 patients from 14 kindreds with severe hypertriglyceridemia (>1000 mg/dl).
Patients were evaluated over 12.5 years, including clinical assessment, genetic screening, and dietary intervention.
Dietary modification involved a stringent fat-restricted diet.

Main Results:

Common clinical findings included hepatomegaly, splenomegaly, and lipemia retinalis.
A fat-restricted diet led to dramatic improvement in fasting lipid profiles in 14 out of 15 compliant patients.
Genetic screening identified six different variants, including two novel ones.

Conclusions:

Lipemic serum, chylothorax, and recurrent pancreatitis in children warrant suspicion for LPL deficiency.
Early diagnosis and prompt initiation of a stringent fat-restricted diet are key for successful management and pancreatitis prevention.