Navigation Links
ABCB6 is key to production of heme in hemoglobin

Investigators at St. Jude Children's Research Hospital have discovered that a protein called ABCB6 plays a central role in production of a molecule that is key to the ability of red blood cells to carry oxygen, of liver cells to break down toxins, and of cells to extract energy from nutrients.

The St. Jude investigators showed that ABCB6 is lodged within the outer membrane of the cell's energy powerhouse called the mitochondria; and that it ferries into mitochondria a type of molecule called a porphryin, a molecule essential for life. A report on these results appears in the advanced online publication of Nature.

Inside the mitochondrion, porphyrins are converted to heme. Heme is the oxygen-carrying part of the red blood cell molecule called hemoglobin, as well as a critical part of certain liver enzymes that break down toxins, and so-called "respiratory chain" enzymes in mitochondria that use oxygen to produce energy-rich molecules.

The discovery of the location and function of ABCB6 solved the long-standing riddle of how porphyrins get into mitchondria so they can be used to make heme, said John Schuetz, PhD, a member of St. Jude Pharmaceutical Sciences. "Porphyrins are negatively charged and so is the inside of mitochondria," Schuetz said. "So it wasn't clear how a negatively charged molecule could get into a negatively charged environment when it should have been repulsed--like two negative poles of a magnet pushing each other apart. Our work showed that ABCB6 overcomes that problem by simply grabbing porphyrin molecules and pulling them in.

"We showed that there is a dynamic relationship between ABCB6 and the levels of heme and porphyrin that helps to regulate the production of heme," Schuetz said. The team found that the increase in ABCB6 in mitochondrial membranes caused the cells to make more porphyrin, the building block of heme. "Any disruption of this relationship can cause serious problems in the cell by altering leve ls of heme or porphyrins," Schuetz said.

Armed with this new information, researchers will be better able to study certain diseases at the molecular level and learn more about their causes and perhaps how to prevent or treat them. One such group of diseases is cutaneous porphyria, a buildup of porphyrins that causes the skin to blister and swell in sunlight; and acute porphoryia, a nerve problem that triggers pain in the chest, abdomen, limbs or back, as well as paralysis, cramping and personality changes or mental disorders. "Also, people with porphyria can suffer sudden, acute attacks if they take certain tranquilizers and other drugs that interfere with this system," Schuetz said. "So our work contributes to the overall picture of how disruption of the biochemical pathway of heme production can cause a variety of serious diseases."


'"/>

Source:St. Jude Children's Research Hospital


Related biology news :

1. Researchers discover key gene involved in bark beetle pheromone production
2. First production of human monoclonal antibodies in chicken eggs published in Nature Biotechnology
3. Mass production of human papillomavirus could lead to gains against cervical cancer
4. UW scientists report a new method to speed bird flu vaccine production
5. New cell transplantation technique restores insulin production in diabetics
6. New molecule may aid in production of biofuels and fungi-resistant plants
7. Getting an evolutionary handle on life after reproduction
8. Olfactory system detects pheromones that control reproduction
9. The brain is broadly wired for reproduction
10. Sexual reproduction delays aging in a mammalian species
11. Sustainable farm practices improve Third World food production
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:7/20/2017)... -- Delta (NYSE: DAL ) customers now can use fingerprints ... Washington National Airport (DCA). ... Delta launches biometrics to board aircraft at Reagan Washington National ... Delta,s biometric boarding pass experience that launched ... into the boarding process to allow eligible Delta SkyMiles Members who are ...
(Date:5/16/2017)...  Veratad Technologies, LLC ( www.veratad.com ), an innovative ... verification solutions, announced today they will participate as a ... thru May 17, 2017, in Washington D.C.,s ... Identity impacts the lives of billions ... evolving digital world, defining identity is critical to nearly ...
(Date:4/18/2017)... 18, 2017  Socionext Inc., a global expert in SoC-based imaging ... server, the M820, which features the company,s hybrid codec technology. A ... Tera Probe, Inc., will be showcased during the upcoming Medtec Japan ... at the Las Vegas Convention Center April ... Click here for ...
Breaking Biology News(10 mins):
(Date:10/11/2017)... ... October 11, 2017 , ... ... in its endogenous context, enabling overexpression experiments and avoiding the use of exogenous ... RNA guides is transformative for performing systematic gain-of-function studies. , This complement ...
(Date:10/11/2017)... ... October 11, 2017 , ... Proscia Inc ., a ... Webinar titled, “Pathology is going digital. Is your lab ready?” with Dr. Nicolas ... practices and how Proscia improves lab economics and realizes an increase in diagnostic ...
(Date:10/11/2017)... ... 2017 , ... Disappearing forests and increased emissions are the main causes of ... year. Especially those living in larger cities are affected by air pollution related diseases. ... most pollution-affected countries globally - decided to take action. , “I knew I had ...
(Date:10/10/2017)... ... 10, 2017 , ... San Diego-based team building and cooking events company, Lajollacooks4u, ... The bold new look is part of a transformation to increase awareness, appeal ... growth period. , It will also expand its service offering from its signature gourmet ...
Breaking Biology Technology: