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Related Concept Videos

Gap Junctions01:27

Gap Junctions

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The cytoplasm of adjacent animal cells can exchange small molecules, ions, and secondary messengers via the communication channels which form the gap junctions. These junctions comprise a few hundred to thousands of molecular channels, each made of two halves, called the connexon hemichannel. A connexon is a hexamer of six transmembrane connexin proteins, which assemble radially, thus forming a pore or channel in the center. One connexon hemichannel docks with a corresponding connexon on the...
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Gap Junctions01:37

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Multicellular organisms employ a variety of ways for cells to communicate with each other. Gap junctions are specialized proteins that form pores between neighboring cells in animals, connecting the cytoplasm between the two, and allowing for the exchange of molecules and ions. They are found in a wide range of invertebrate and vertebrate species, mediate numerous functions including cell differentiation and development, and are associated with numerous human diseases, including cardiac and...
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Local Anesthetics: Differential Sensitivity of Nerve Fibers01:24

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Local anesthetics (LAs) block the sodium channels of nerve trunks, sensory nerve endings, and neuromuscular junctions. Although LAs can block all kinds of nerves, the sensitivity of nerve fibers differs according to nerve types and structures. LAs are known to block myelinated fibers faster than unmyelinated ones. Also, they block pain or sensory neurons at low concentrations without affecting the motor neurons involved in muscle contractions. This helps relieve labor pain without affecting the...
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Contact-dependent Signaling01:19

Contact-dependent Signaling

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Contact-dependent signaling, as the name suggests, requires that communicating cells be in direct contact with each other. This is achieved either through receptor-ligand interactions or by specialized cytoplasmic channels that allow the flow of small molecules between cells. In animal cells, channels called gap junctions facilitate contact-dependent signaling in certain tissues, whereas, plasmodesmata perform a similar function in plants.
Gap Junctions
In animal cells, gap junctions are formed...
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Diabetic Neuropathy01:22

Diabetic Neuropathy

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DefinitionDiabetic neuropathy is nerve damage caused by long-standing diabetes mellitus. It results directly from prolonged high blood sugar levels.PathophysiologyThe pathophysiology of diabetic neuropathy involves both metabolic and vascular disturbances triggered by chronic hyperglycemia.Metabolic injury: Elevated glucose levels activate the polyol pathway within nerve cells, leading to the accumulation of sorbitol and fructose. This increases oxidative stress, disrupts normal nerve...
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Peripheral Nervous System: Ganglia and Nerves01:24

Peripheral Nervous System: Ganglia and Nerves

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The Peripheral Nervous System (PNS) is a crucial component of the body's neural network, extending beyond the central nervous system (CNS) to bridge the gap between the CNS and the external environment. It encompasses nerves, ganglia, and sensory receptors.
Nerves
The nerve is a bundle of axons that serves as the communication highway in the PNS. Each nerve is ensheathed in a protective layer of connective tissue called the epineurium. This outermost layer safeguards the nerve and supports the...
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An Iodide-Yellow Fluorescent Protein-Gap Junction-Intercellular Communication Assay
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Connexins, gap junctions and peripheral neuropathy.

Kleopas A Kleopa1, Irene Sargiannidou2

  • 1Neurology Clinics, The Cyprus Institute of Neurology and Genetics and Cyprus School of Molecular Medicine, Nicosia, Cyprus; Neuroscience Laboratory, The Cyprus Institute of Neurology and Genetics and Cyprus School of Molecular Medicine, Nicosia, Cyprus.

Neuroscience Letters
|December 3, 2014
PubMed
Summary
This summary is machine-generated.

Mutations in connexin32 (Cx32) cause X-linked Charcot-Marie-Tooth neuropathy (CMT1X), a peripheral nerve disease. Understanding Cx32 gap junction dysfunction is key to developing therapies for this incurable condition.

Keywords:
Cx32Myelinated fibersSchwann cellsX-linked Charcot–Marie–Tooth disease (CMT1X)

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Gap junctions (GJs) formed by connexin32 (Cx32) are crucial for peripheral myelinated nerve function.
  • Mutations in the GJB1 gene encoding Cx32 cause X-linked Charcot-Marie-Tooth neuropathy (CMT1X), a common peripheral neuropathy.

Purpose of the Study:

  • To explore the role of Cx32 in peripheral nerve homeostasis and CMT1X pathogenesis.
  • To understand how Cx32 mutations lead to loss of GJ function and neuropathy.

Main Methods:

  • Review of existing literature on Cx32, GJs, and CMT1X.
  • Analysis of in vitro and in vivo models of Cx32 mutations.

Main Results:

  • Over 400 GJB1 mutations identified in CMT1X patients.
  • Most Cx32 mutations result in loss of GJ function.
  • Cx32 GJs facilitate ion and molecule diffusion critical for myelinated axon health.

Conclusions:

  • Cx32-formed gap junctions are vital for maintaining myelinated axon integrity.
  • Dysfunctional Cx32 gap junctions are central to CMT1X pathogenesis.
  • Further research into CMT1X pathogenesis can guide therapeutic strategies.