Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Cooperative Binding of Transcription Regulators02:13

Cooperative Binding of Transcription Regulators

7.3K
Transcriptional regulators bind to specific cis-regulatory sequences in the DNA to regulate gene transcription. These cis-regulatory sequences are very short, usually less than ten nucleotide pairs in length. The short length means that there is a high probability of the exact same sequence randomly occurring throughout the genome.  Since regulators can also bind to groups of similar sequences, this further increases the chances of random binding. Transcriptional regulators form...
7.3K
Cooperative Binding of Transcription Regulators02:13

Cooperative Binding of Transcription Regulators

2.6K
2.6K
Regulated Protein Degradation02:58

Regulated Protein Degradation

8.8K
It is vital to regulate the activity of enzymatic as well as non-enzymatic proteins inside the cell. This can be achieved either through creating a balance between their rate of synthesis and degradation or regulating the intrinsic activity of the protein. Both these regulation mechanisms play an essential role in the normal functioning of cells.
Protein degradation plays two important roles in the cells. It helps to protect cells from misfolded or damaged proteins before they lead to a...
8.8K
Covalently Linked Protein Regulators02:04

Covalently Linked Protein Regulators

9.5K
Proteins can undergo many types of post-translational modifications, often in response to changes in their environment. These modifications play an important role in the function and stability of these proteins. Covalently linked molecules include functional groups, such as methyl, acetyl, and phosphate groups, and also small proteins, such as ubiquitin. There are around 200 different types of covalent regulators that have been identified.
These groups modify specific amino acids in a protein....
9.5K
GTPases and their Regulation02:14

GTPases and their Regulation

9.8K
Guanine nucleotide-binding proteins (G-proteins), also known as GTPases, are a superfamily of proteins that regulate many cellular processes, such as cell signaling, vesicular transport, and the regulation of cell shape and motility. Mutation or dysfunction of these proteins can lead to disease. There are around 40,000 known G-proteins that can broadly be classified into two groups ‒  small G-proteins consisting of a single domain and large multi-domain G-proteins.
Large G-proteins,...
9.8K
Master Transcription Regulators02:23

Master Transcription Regulators

7.8K
Master transcription regulators are regulatory proteins that are predominantly responsible for regulating the expression of multiple genes. Often these genes work in concert to drive a  complex process. Activation of a master transcription regulator can lead to a cascade of transcriptional activation necessary for that outcome. These regulators can directly bind to the regulatory sequences of the various genes involved, or they can indirectly regulate transcription by binding to regulatory...
7.8K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Prion-like transmission and propagation of human β-amyloid to the bank vole rodent model.

Acta neuropathologica·2026
Same author

Burden and trends of mental disorders in Vietnam, 1990-2023: insights from the Global Burden of Disease Study 2023.

Osong public health and research perspectives·2026
Same author

Multi-target approaches to prion disease drug discovery: a status update.

Expert opinion on drug discovery·2026
Same author

Optical Tweezers in Emulsion Research: Principles, Advances, and Prospects.

Langmuir : the ACS journal of surfaces and colloids·2026
Same author

Detection of TDP-43 seeds in CSF of presymptomatic and symptomatic genetic FTD/ALS.

Alzheimer's & dementia : the journal of the Alzheimer's Association·2025
Same author

How Sup35 monomer conformation and amyloid fibril polymorphism determine yeast strain phenotypes.

Research square·2025
Same journal

Exerkine-Mediated Regulation of the NLRP3 Inflammasome in Neuroprotection: Mechanistic Insights and the Role of Exercise.

Molecular neurobiology·2026
Same journal

miR-6836-5p Drives Astrocyte Pro-survival Signaling Through DLG2-Hippo-YAP Pathway Under AQP4-IgG + ve NMOSD Stress.

Molecular neurobiology·2026
Same journal

Nrf2 Activators in Parkinson's Disease: Modulating Mitophagy and Regulating Cuproptosis.

Molecular neurobiology·2026
Same journal

The Molecular Machinery of Synaptic Plasticity and Its Potential Role in the Aetiology of Schizophrenia.

Molecular neurobiology·2026
Same journal

Hypothalamus Amyloid Levels Are Associated with Early Sex-Dependent Alterations in Peripheral Energy Homeostasis in TgF344-AD Rats.

Molecular neurobiology·2026
Same journal

A Dose-Response Study on Human FGF21 to Inhibit Necrosis, Spectrin Breakdown, and to Increase Intracellular Cold Shock Proteins in Cultured Cortical Neurons Subjected to Oxygen-Glucose Deprivation Injury.

Molecular neurobiology·2026
See all related articles

Related Experiment Video

Updated: Jan 29, 2026

Detection of Abnormal Prion Protein by Immunohistochemistry
06:38

Detection of Abnormal Prion Protein by Immunohistochemistry

Published on: May 5, 2023

3.7K

Copper Binding Regulates Cellular Prion Protein Function.

Xuan T A Nguyen1, Thanh Hoa Tran1, Dan Cojoc2

  • 1Laboratory of Prion Biology, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy.

Molecular Neurobiology
|February 8, 2019
PubMed
Summary
This summary is machine-generated.

The cellular prion protein (PrPC) uses N-terminal copper-binding sites to promote neuron growth. Disrupting these sites impairs this function and can be toxic, highlighting their importance in neuronal development and potentially disease.

Keywords:
Copper-binding siteGrowth coneNeuritogenesisPrion protein

More Related Videos

Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

20.2K
Identification of Small Molecule-binding Proteins in a Native Cellular Environment by Live-cell Photoaffinity Labeling
10:49

Identification of Small Molecule-binding Proteins in a Native Cellular Environment by Live-cell Photoaffinity Labeling

Published on: September 20, 2016

13.3K

Related Experiment Videos

Last Updated: Jan 29, 2026

Detection of Abnormal Prion Protein by Immunohistochemistry
06:38

Detection of Abnormal Prion Protein by Immunohistochemistry

Published on: May 5, 2023

3.7K
Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

20.2K
Identification of Small Molecule-binding Proteins in a Native Cellular Environment by Live-cell Photoaffinity Labeling
10:49

Identification of Small Molecule-binding Proteins in a Native Cellular Environment by Live-cell Photoaffinity Labeling

Published on: September 20, 2016

13.3K

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Prion Biology

Background:

  • The cellular prion protein (PrPC) is implicated in neurodegenerative diseases and physiological processes like neuritogenesis.
  • PrPC is known to bind copper and zinc, suggesting a role in metal homeostasis.
  • The precise impact of copper on PrPC's physiological functions remains largely unexplored.

Purpose of the Study:

  • To investigate how copper binding influences the neuritogenesis function of PrPC.
  • To elucidate the role of N-terminal copper-binding sites in PrPC-mediated neuronal growth.
  • To explore the potential link between PrPC function, copper binding, and disease initiation.

Main Methods:

  • Utilized genomic approaches, cellular assays, and focal stimulation techniques.
  • Generated recombinant mouse PrP (recMoPrP) mutants with altered N-terminal copper-binding sites.
  • Applied bulk and focal stimulation methods to primary hippocampal cultures and neuronal growth cones (GC).

Main Results:

  • Focal stimulation with wild-type recMoPrP induced neurite outgrowth and growth cone turning.
  • recMoPrP mutants lacking functional N-terminal copper-binding sites failed to support neuritogenesis.
  • Complete disruption of N-terminal copper-binding sites proved toxic to neurons, underscoring their essential role.

Conclusions:

  • Copper-binding sites at the N-terminus of PrPC are critical for its neuritogenesis function.
  • Both octarepeat and non-octarepeat copper-binding regions are essential for this effect.
  • Dysfunction of these copper-binding sites may contribute to disease initiation through loss-of-function mechanisms.