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Related Concept Videos

Disassembly of Intermediate Filaments01:35

Disassembly of Intermediate Filaments

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Intermediate filaments (IFs) do not undergo spontaneous disassembly. Enzymes, kinases, and phosphatases add and remove phosphates from specific sites to regulate their disassembly. The IF concentration in the cytoplasm also regulates the disassembly. If the concentration crosses a threshold, it activates the protein kinases in the vicinity, allowing the phosphorylation of IFs.
Keratin proteins, found at the cell periphery near cell junctions, undergo a cycle of assembly and disassembly. In Type...
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Types of Intermediate Filaments01:31

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The intermediate filaments are an essential component of the cytoskeleton. Presently six types of intermediate filament have been identified. Type I and II are acidic and basic keratin proteins. Type III is of mesodermal origin and comprises four proteins: vimentin, desmin, glial fibrillary acidic protein (GFAP), and peripherin. Vimentin is commonly found in mesenchymal cells, desmin in muscle cells, GFAP in astrocytes, while peripherin is found in peripheral nervous system neurons (PNS). Type...
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Formation of Intermediate Filaments00:57

Formation of Intermediate Filaments

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Intermediate filaments are cytoskeletal proteins with higher tensile strength and flexibility than microfilaments and microtubules. Unlike the other two cytoskeletal proteins, intermediate filament formation lacks the enzymatic activity to hydrolyze nucleotides like ATP and GTP to generate energy for polymerization. Therefore, the formation of intermediate filaments is multistep self-assembly. The involvement of any accessory proteins in intermediate filament formation has not yet been...
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The Structure of Intermediate Filaments01:19

The Structure of Intermediate Filaments

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The intermediate filaments are one of three widely studied cytoskeletal filaments. They are so named as their diameter (10 nm) is in between that of microfilaments (7 nm) and the microtubules (25 nm).  These filaments are highly stable and can remain intact when exposed to high salt concentrations and detergents. These filaments are responsible for providing stability and mechanical support to the cells. They also help in cell adhesion and maintaining tissue integrity.
Intermediate...
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Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

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Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Imaging Intermediate Filaments and Microtubules with 2-dimensional Direct Stochastic Optical Reconstruction Microscopy
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Intermediate filaments in cardiomyopathy.

Mary Tsikitis1, Zoi Galata1, Manolis Mavroidis1

  • 1Center of Basic Research, Biomedical Research Foundation, Academy of Athens, 4 Soranou Ephesiou, 11527, Athens, Greece.

Biophysical Reviews
|July 21, 2018
PubMed
Summary
This summary is machine-generated.

Intermediate filament (IF) proteins are vital for heart function. Disruptions in IFs like desmin and lamin cause cardiomyopathies and heart failure by impairing cellular processes.

Keywords:
CardiomyopathiesDesminDesminopathiesDilated cardiomyopathyHeart failureIntermediate filamentsLaminLaminopathiesMitochondrial defects

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Cardiology

Background:

  • Intermediate filament (IF) proteins are crucial for cellular structure and function.
  • Mutations and modifications in IF genes are associated with numerous human diseases.
  • Desmin and lamin are key IF proteins implicated in cardiac health and disease.

Purpose of the Study:

  • To review the role of IF proteins in cardiac function.
  • To explore how IF disruptions lead to cardiomyopathy and heart failure.

Main Methods:

  • Literature review of studies on IF proteins in healthy and diseased hearts.
  • Analysis of the impact of IFs on cellular processes like mechanotransduction and metabolism.

Main Results:

  • IF networks are essential for structural integrity, mechanotransduction, gene activation, and cardiomyocyte survival.
  • Proper IF function is critical for mitochondrial homeostasis and metabolic regulation.
  • Deficiency or disruption of IFs significantly contributes to cardiomyopathy and heart failure.

Conclusions:

  • Intermediate filaments play a critical role in maintaining cardiac structure and function.
  • Dysregulation of IFs, particularly desmin and lamin, is a significant factor in the development of heart failure.