Related Experiment Video

Updated: Apr 27, 2026

Cell Membrane Repair Assay Using a Two-photon Laser Microscope
06:35

Cell Membrane Repair Assay Using a Two-photon Laser Microscope

Published on: January 2, 2018

12.3K

Dysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion

A Defour1, J H Van der Meulen1, R Bhat1

  • 1Center for Genetic Medicine Research, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC, USA.

Cell Death & Disease
|June 27, 2014
PubMed

Insights

Dysferlin deficiency impairs muscle membrane repair by reducing lysosomal exocytosis. Restoring acid sphingomyelinase (ASM) activity can rescue this defect, offering a potential therapy for dysferlinopathy.

Area of Science:

  • Muscle biology
  • Cellular repair mechanisms
  • Lysosomal function

Background:

  • Dysferlin deficiency impairs skeletal muscle membrane repair, but the cellular mechanisms are not fully understood.
  • Dysferlinopathy affects muscle regeneration and function, highlighting the need to elucidate its molecular basis.

Purpose of the Study:

  • To investigate the cellular mechanism of impaired muscle membrane repair in dysferlinopathy.
  • To identify potential therapeutic targets for dysferlinopathy.

Main Methods:

  • Developed mouse and human myoblast models of dysferlinopathy.
  • Utilized live-cell imaging to observe membrane repair processes.
  • Assessed lysosomal exocytosis and acid sphingomyelinase (ASM) secretion in response to injury.

Main Results:

  • Dysferlin-deficient myoblasts showed reduced lysosomal exocytosis at the cell membrane following injury.
  • Individual lysosomes, not lysosome-lysosome fusion, mediate membrane repair through fusion with the sarcolemma.
  • Reduced ASM secretion was observed in injured dysferlinopathic cells; exogenous ASM treatment restored repair capacity.

Conclusions:

  • Dysferlin is crucial for efficient lysosomal exocytosis, a key mechanism in skeletal muscle sarcolemma repair.
  • Acid sphingomyelinase (ASM) plays a vital role in this repair process.
  • ASM represents a promising therapeutic target for treating dysferlinopathy.

More Related Videos

Live Imaging Assay for Assessing the Roles of Ca2+ and Sphingomyelinase in the Repair of Pore-forming Toxin Wounds18:25

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

Published on: August 25, 2013

11.1K
High-throughput Measurement of Plasma Membrane Resealing Efficiency in Mammalian Cells10:07

High-throughput Measurement of Plasma Membrane Resealing Efficiency in Mammalian Cells

Published on: January 7, 2019

7.1K

Related Concept Videos

Enlargement of the Plasma Membrane01:22

Enlargement of the Plasma Membrane

Cell division and enlargement are processes that require precise control. The control ensures that cell division cannot proceed unless the cell has grown to a specific size. A spherical, dividing cell requires an approximately 1.6X increase in its surface area to double its volume. The secretory pathway also has a significant role in cell membrane enlargement. Secretory vesicles that bud off from the Golgi apparatus and later fuse with the plasma membrane during exocytosis are a major source of...
1.8K
Assembly of the Lipid Bilayer in the ER01:28

Assembly of the Lipid Bilayer in the ER

Biological membranes are more than just a barrier separating cell cytoplasm from the outside environment. They are highly dynamic and help maintain the integrity and physiological stability of the cells as well as membrane-bound organelles. Membranes also play vital roles in cell-to-cell and intracellular communication.
A large chunk of any biological membrane is composed of phospholipids. These lipids have a heterogeneous distribution across different subcellular organelles and even between...
3.2K
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%...
8.0K
Desmosomes01:05

Desmosomes

The term desmosome derives from the Greek words "desmo" and "soma" meaning "adhesion bodies." This structure was first observed during the late 1800s and described as small, dense nodules in the epidermis. Desmosomes are button-like structures that help form an interlinked network of intermediate filaments across the cells. These junctions are  essential to hold cells together under mechanical stress and to maintain tissue integrity. Desmosomes are multi-protein...
7.4K
Role of Ephrin-Eph Signalling in Intestinal Stem Cell Renewal01:22

Role of Ephrin-Eph Signalling in Intestinal Stem Cell Renewal

Erythropoietin-producing hepatocellular carcinoma receptor (Eph) and its ligand, Eph receptor-interacting protein (Ephrin) were first discovered in the human carcinoma cell line, hence the name. Ephrin-Eph interaction guides cells to reach their appropriate location in adult tissues. They also play an essential role in the immune system by helping in immune cell migration, adhesion, and activation. Based on their structure and function, Eph is divided into two classes — EphA and EphB.
1.8K
Cell Motility through Blebbing01:16

Cell Motility through Blebbing

Blebs are a type of membrane protrusion formed by the internal hydrostatic pressure of the cytoplasm. Blebs are observed in several cell types, including fibroblasts, immune cells, and single-celled organisms like the amoeba. The primary function of blebs is cell locomotion and apoptosis, but they are also found during necrosis and cell division. The life cycle of a bleb comprises an initiation phase followed by the expansion and retraction phases.
Blebbing Through the Matrix
In multicellular...
1.9K