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Related Concept Videos

Membrane Lipids01:32

Membrane Lipids

Lipids are an essential component of all biological membranes. The average lipid content in mammalian membranes is 50%, though it can be as low as 20% in the inner mitochondrial membrane or as high as 80% in the myelin sheath present around the nerve cells.
Phosphatidylcholine, phosphatidylethanolamine, phosphatidylserine, and sphingomyelin are the most common phospholipids present in mammalian membranes. At physiological pH, phosphatidylserine is negatively charged, while the other three...
Asymmetric Lipid Bilayer01:35

Asymmetric Lipid Bilayer

Biological membranes show uneven distribution of different types of lipids in the inner and outer layers, resulting in transverse asymmetric membranes. The treatment of the erythrocyte membrane with the enzyme phospholipase confirmed the asymmetric nature of the lipid bilayer. The enzyme hydrolyzes lipids into fatty acids and hydrophilic groups. The phospholipase acts only on the outer layer of the membrane, while the inner layer remains intact. The phospholipase treatment resulted in 80%...
Membrane Domains01:18

Membrane Domains

The membrane domains concentrate specific lipids and proteins at one place within the membrane, which helps in cell signaling, adhesion, and other critical cellular processes. These domains can differ in size, composition, function, and lifespan.
Protein Domains
The membrane comprises a group of distinct proteins responsible for carrying out a cell's specific function. For example, the plasma membrane of the human sperm, or a single germ cell, contains a unique set of proteins in the anterior...
Introduction to Membrane Traffic01:44

Introduction to Membrane Traffic

The ER, Golgi apparatus, endosomes, and lysosomes work in tandem to modify, sort, and package proteins and lipids. An integrated membrane trafficking network facilitates the back and forth shuttling of molecules within different organelles in the same cell or across the cell membrane.
The transport of soluble and membrane proteins is mediated by transport vesicles that collect cargo from one cellular compartment and deliver it to another by fusing with the target organelle membrane. The Rab...
Lysosomal Hydrolases01:22

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,...
Membrane Asymmetry Regulating Transporters01:19

Membrane Asymmetry Regulating Transporters

Enzymes like flippase, floppase, and scramblase transfer phospholipids from one layer to another in the membrane, thereby affecting membrane asymmetry.
Flippase
Eukaryotic flippases are type-IV P-type ATPases or P4-ATPases belonging to P-type ATPase family proteins that are membrane-bound pumps involved in the ATP-mediated transport of ions and molecules across the membrane. Flippases flip specific phospholipids from the outer to the inner leaflet of a membrane. All P4-ATPases have one...

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Related Experiment Video

Updated: Jul 23, 2026

Live Imaging Assay for Assessing the Roles of Ca2+ and Sphingomyelinase in the Repair of Pore-forming Toxin Wounds
18:25

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Published on: August 25, 2013

Membrane traffic in sphingolipid storage diseases.

R E Pagano1, V Puri, M Dominguez

  • 1Department of Biochemistry and Molecular Biology, Mayo Clinic and Foundation, 200 First Street, S.W., Rochester, MN 55905, USA. pagano.richard@mayo.edu

Traffic (Copenhagen, Denmark)
|February 24, 2001
PubMed
Summary

Sphingolipid storage diseases (SLSDs) cause lipid buildup in cells. Our research shows that altered cholesterol distribution due to this buildup disrupts sphingolipid transport, offering new therapeutic targets.

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Area of Science:

  • Cell Biology
  • Biochemistry
  • Genetics

Background:

  • Sphingolipids (SLs) are crucial membrane components.
  • Sphingolipid storage diseases (SLSDs) result from defects in lipid metabolism.
  • Altered lipid transport is a hallmark of SLSDs.

Purpose of the Study:

  • To investigate membrane lipid transport in SLSD fibroblasts.
  • To elucidate the role of cholesterol in SL mistargeting.
  • To propose a new model for lipid traffic regulation in SLSDs.

Main Methods:

  • Utilized fluorescent sphingolipid analogs for uptake studies.
  • Examined lipid distribution in normal and SLSD fibroblasts.
  • Assessed cholesterol homeostasis in various SLSD cell types.

Main Results:

  • Normal cells target fluorescent SL analogs to the Golgi complex.
  • Ten SLSD cell types showed accumulation of SL analogs in endosomes/lysosomes.
  • Cholesterol homeostasis was perturbed in SLSDs, regulating SL analog mistargeting.

Conclusions:

  • Endogenous SL accumulation in SLSDs alters intracellular cholesterol distribution.
  • This alteration leads to defective sorting and transport of SLs.
  • Findings suggest a novel paradigm for endocytic pathway lipid traffic regulation.