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Related Concept Videos

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 complexes comprising desmosomal...
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...
Role of Septins01:02

Role of Septins

Septins are the recently discovered fourth major protein component of the cytoskeleton, along with microfilaments, microtubules, and intermediate filaments. These proteins can associate with other cytoskeletal filaments and carry out varied roles or can be free-floating in the cytoplasm.
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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...
Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
Long-patch Base Excision Repair01:02

Long-patch Base Excision Repair

Since the discovery of the two BER pathways, there has been a debate about how a cell chooses one pathway over the other and the factors determining this selection. Numerous in vitro experiments have pointed out multiple determinants for the sub-pathway selection. These are:

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Related Experiment Video

Updated: Jul 14, 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

Dysferlin in membrane trafficking and patch repair.

Louise Glover1, Robert H Brown

  • 1Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Harvard Medical School, Charlestown, MA 02129, USA.

Traffic (Copenhagen, Denmark)
|June 6, 2007
PubMed
Summary

Dysferlin is crucial for muscle membrane repair after injury, acting as a calcium-regulated fusogen. Defects in this repair mechanism may cause a new class of muscular dystrophies.

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Area of Science:

  • Muscle cell biology
  • Molecular genetics
  • Cell membrane repair mechanisms

Background:

  • Muscular dystrophies are inherited disorders causing progressive muscle weakness and atrophy.
  • Dystrophin and its associated proteins are vital for muscle cell membrane integrity.
  • A new class of muscular dystrophies may result from impaired muscle membrane repair.

Purpose of the Study:

  • To review the role of dysferlin in muscle membrane repair.
  • To explore dysferlin-related signaling pathways in muscle physiology and pathology.

Main Methods:

  • Literature review of studies on muscular dystrophies, dystrophin, and dysferlin.
  • Analysis of the molecular mechanisms of membrane resealing.
  • Examination of calcium-dependent exocytosis in cell repair.

Main Results:

  • Dysferlin is identified as a key component of a muscle-specific membrane repair complex.
  • Dysferlin facilitates rapid resealing of mechanically disrupted muscle membranes.
  • Dysferlin's function resembles calcium-regulated exocytosis, potentially acting as a fusogen.

Conclusions:

  • Dysferlin plays a critical role in maintaining muscle membrane integrity through repair.
  • Dysferlin-related pathways are central to muscle cell function and disease.
  • Understanding dysferlin's role opens new avenues for investigating muscular dystrophies.