Featured Research

from universities, journals, and other organizations

Iron Is Involved In Prion Disease-associated Neuronal Demise

Date:
March 20, 2009
Source:
Public Library of Science
Summary:
Imbalance of iron homeostasis is a common feature of prion disease-affected human, mouse, and hamster brains, according to a new study. These findings provide new insight into the mechanism of neurotoxicity in prion disorders, and novel avenues for the development of therapeutic strategies.

Imbalance of iron homeostasis is a common feature of prion disease-affected human, mouse, and hamster brains, according to a new study by Dr. Neena Singh and colleagues at Case Western Reserve University School of Medicine, alongside collaborators from Creighton University.

These findings provide new insight into the mechanism of neurotoxicity in prion disorders, and novel avenues for the development of therapeutic strategies.

Unlike other neurodegenerative conditions, prion disorders are sporadic, inherited, and infectious, and affect both humans and animals; common examples are mad cow disease in cattle, scrapie in sheep, and Creutzfeldt-Jakob disease in humans. The causative agent is a misfolded protein referred to as PrP-scrapie that replicates itself by changing the conformation of neighboring copies of the same protein, namely the prion protein. Aggregates of PrP-scrapie are toxic to brain cells and cause a spongy-like appearance in diseased brains.

Research from the Singh laboratory suggests that accumulation of PrP-scrapie alters the metabolism of iron in diseased brains. The imbalance of brain iron homeostasis worsens with disease progression, and is not an outcome of end-stage disease. Since iron is highly toxic when mismanaged, this condition is likely to contribute significantly to prion-disease-associated neurotoxicity. The likely cause of this condition is loss of normal function of the prion protein in cellular iron metabolism demonstrated recently by Singh and colleagues, combined with gain of toxic function by the redox-active PrP-scrapie complex as shown in this report.

Singh and her team were surprised to find that prion disease-affected brains are iron deficient despite a significant increase in their overall iron content. The group concludes that ferritin, a major iron storage protein, co-aggregates with PrP-scrapie in diseased brains and sequesters bound iron in the complex, creating a state of apparent iron deficiency. The brain cells respond to this condition by increasing their level of iron uptake, thus creating a vicious cycle of increased iron uptake in the presence of increased iron.

These observations contribute to our understanding of how the prion agent causes neurotoxicity, and may enable the development of novel therapeutic strategies targeted at restoring brain iron homeostasis in prion disorders.


Story Source:

The above story is based on materials provided by Public Library of Science. Note: Materials may be edited for content and length.


Journal Reference:

  1. Singh et al. Abnormal Brain Iron Homeostasis in Human and Animal Prion Disorders. PLoS Pathogens, 2009; 5 (3): e1000336 DOI: 10.1371/journal.ppat.1000336

Cite This Page:

Public Library of Science. "Iron Is Involved In Prion Disease-associated Neuronal Demise." ScienceDaily. ScienceDaily, 20 March 2009. <www.sciencedaily.com/releases/2009/03/090313145954.htm>.
Public Library of Science. (2009, March 20). Iron Is Involved In Prion Disease-associated Neuronal Demise. ScienceDaily. Retrieved August 21, 2014 from www.sciencedaily.com/releases/2009/03/090313145954.htm
Public Library of Science. "Iron Is Involved In Prion Disease-associated Neuronal Demise." ScienceDaily. www.sciencedaily.com/releases/2009/03/090313145954.htm (accessed August 21, 2014).

Share This




More Plants & Animals News

Thursday, August 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Possible Ebola Patient in Isolation at California Hospital

Possible Ebola Patient in Isolation at California Hospital

Reuters - US Online Video (Aug. 20, 2014) — A patient who may have been exposed to the Ebola virus is in isolation at the Kaiser Permanente South Sacramento Medical Center. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Flower Power! Dandelions Make Car Tires?

Flower Power! Dandelions Make Car Tires?

Reuters - Business Video Online (Aug. 20, 2014) — Forget rolling on rubber, could car drivers soon be traveling on tires made from dandelions? Teams of scientists are racing to breed a type of the yellow flower whose taproot has a milky fluid with tire-grade rubber particles in it. As Joanna Partridge reports, global tire makers are investing millions in research into a new tire source. Video provided by Reuters
Powered by NewsLook.com
Unsustainable Elephant Poaching Killed 100K In 3 Years

Unsustainable Elephant Poaching Killed 100K In 3 Years

Newsy (Aug. 20, 2014) — Poachers have killed 100,000 elephants between 2010 and 2012, as the booming ivory trade takes its toll on the animals in Africa. Video provided by Newsy
Powered by NewsLook.com
Awesome New Camouflage Sheet Was Inspired By Octopus Skin

Awesome New Camouflage Sheet Was Inspired By Octopus Skin

Newsy (Aug. 19, 2014) — Scientists have developed a new device that mimics the way octopuses blend in with their surroundings to hide from dangerous predators. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins