Study sheds new light on role iron biology plays in disease

November 29, 2019

New research shows that the body's system for regulating iron is much more complex than originally thought--and this has surprising implications in at least three human diseases, according to a new study by University of Alberta biologists.

"Having too much or too little iron can be very damaging for our cells. The bottom line is, every cell needs to have strict control over iron levels," said Kirst King-Jones, associate professor in the University of Alberta's Department of Biological Sciences and senior author on the study.

Nearly every living cell on Earth requires iron to survive. In humans, iron is responsible for transporting oxygen in the blood through hemoglobin. It is also involved with the production of steroid hormones, such as estrogen and testosterone, DNA repair, and many other enzyme reactions. "Because it is so versatile, regulating iron in our cells is extremely important," added King-Jones.

A key component of the iron regulation system, a protein called iron-regulatory protein, can switch between two functions. When iron levels are normal, the protein is bound to a type of iron cofactor, commonly known as an iron-sulphur cluster, and in this form the protein is called aconitase. However, when iron levels drop, the protein loses the iron-sulphur cluster, which allows it to regulate specific genes important for the cell's iron levels.

Previously, the aconitase form was not thought to have any role in iron regulation, but the new research indicates that aconitase has a previously undiscovered role in iron regulation. In fact, the study shows that the aconitase regulates genes in the nucleus of the cell, rather than acting as a superfluous enzyme outside the nucleus.

"Our results demonstrate that three diseases--porphyria (also known as vampire disease), Andersen disease, and Wolfram syndrome--are affected by the function of the aconitase and the level of iron in our cells," said King-Jones. "These results have potential implications for the management of these diseases, through managing iron levels."

The research identified two other key components in the molecular iron regulation system, glycogen-branching enzyme and a protein called mitoNEET, both of which play a role in repairing damage to the iron-regulatory protein.
The paper, "Glycogen branching enzyme controls cellular iron homeostasis via Iron Regulatory Protein 1 and mitoNEET," was published in Nature Communications (doi: 10.1038/s41467-019-13237-8).

University of Alberta

Related Science Articles from Brightsurf:

75 science societies urge the education department to base Title IX sexual harassment regulations on evidence and science
The American Educational Research Association (AERA) and the American Association for the Advancement of Science (AAAS) today led 75 scientific societies in submitting comments on the US Department of Education's proposed changes to Title IX regulations.

Science/Science Careers' survey ranks top biotech, biopharma, and pharma employers
The Science and Science Careers' 2018 annual Top Employers Survey polled employees in the biotechnology, biopharmaceutical, pharmaceutical, and related industries to determine the 20 best employers in these industries as well as their driving characteristics.

Science in the palm of your hand: How citizen science transforms passive learners
Citizen science projects can engage even children who previously were not interested in science.

Applied science may yield more translational research publications than basic science
While translational research can happen at any stage of the research process, a recent investigation of behavioral and social science research awards granted by the NIH between 2008 and 2014 revealed that applied science yielded a higher volume of translational research publications than basic science, according to a study published May 9, 2018 in the open-access journal PLOS ONE by Xueying Han from the Science and Technology Policy Institute, USA, and colleagues.

Prominent academics, including Salk's Thomas Albright, call for more science in forensic science
Six scientists who recently served on the National Commission on Forensic Science are calling on the scientific community at large to advocate for increased research and financial support of forensic science as well as the introduction of empirical testing requirements to ensure the validity of outcomes.

World Science Forum 2017 Jordan issues Science for Peace Declaration
On behalf of the coordinating organizations responsible for delivering the World Science Forum Jordan, the concluding Science for Peace Declaration issued at the Dead Sea represents a global call for action to science and society to build a future that promises greater equality, security and opportunity for all, and in which science plays an increasingly prominent role as an enabler of fair and sustainable development.

PETA science group promotes animal-free science at society of toxicology conference
The PETA International Science Consortium Ltd. is presenting two posters on animal-free methods for testing inhalation toxicity at the 56th annual Society of Toxicology (SOT) meeting March 12 to 16, 2017, in Baltimore, Maryland.

Citizen Science in the Digital Age: Rhetoric, Science and Public Engagement
James Wynn's timely investigation highlights scientific studies grounded in publicly gathered data and probes the rhetoric these studies employ.

Science/Science Careers' survey ranks top biotech, pharma, and biopharma employers
The Science and Science Careers' 2016 annual Top Employers Survey polled employees in the biotechnology, biopharmaceutical, pharmaceutical, and related industries to determine the 20 best employers in these industries as well as their driving characteristics.

Three natural science professors win TJ Park Science Fellowship
Professor Jung-Min Kee (Department of Chemistry, UNIST), Professor Kyudong Choi (Department of Mathematical Sciences, UNIST), and Professor Kwanpyo Kim (Department of Physics, UNIST) are the recipients of the Cheong-Am (TJ Park) Science Fellowship of the year 2016.

Read More: Science News and Science Current Events is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to