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 th e cell by altering levels 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-Newswise
SRM
'"/>




Related medicine news :

1. Therapeutic Monoclonal Antibody Production Is More Profitable Than Small Molecule Drugs
2. Tea Found To Boost Insulin Production And Prevent Cataract
3. Conroy’s May Not Be Permitted To Resume Production Soo
4. Massive Production Of Bird Flu Vaccine To Be Initiated In China
5. Chugai Pharmaceutical To Initiate Domestic Production Of Bird Flu Vaccine
6. Coca Cola To Enhance Production Of Diet Coke
7. Natco Pharma Commences Production At Dehradun Unit
8. New Cell Lines Cuts Down the Cost and Time in the Production of Flu Vaccine
9. Drug Boosts Platelet Production, Reversing Immune Thrombocytopenic Purpura
10. High-Tech Microbes Bolsters Natural Sweetener Production
11. Role of Appetite Hormone MCH in Insulin Production Revealed
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:7/25/2017)... , ... July 25, 2017 , ... ... assistance and finance management services to communities across North Carolina, is launching a ... Network to raise community support for the ongoing fight against bladder cancer. , ...
(Date:7/25/2017)... ... ... Facing medical needs with a newborn boy this spring, Crowley Maritime Corp. ... employees that is uncommon in American business: 12 weeks of fully paid parental leave. ... as new parents. It keeps our family life more stable. I can be there ...
(Date:7/24/2017)... ... ... Cheerag D. Upadhyaya , M.D., M.Sc., FAANS has been appointed surgical ... of Saint Luke’s Health System . Dr. Upadhyaya has served in the role ... joins Stanley P. Fisher, M.D., who has served as medical co-director of ...
(Date:7/24/2017)... ... July 24, 2017 , ... A four alarm fire in ... and threatened numerous homes and businesses nearby, causing some 700 individuals to be ... Angeles Area consulting firm Fire Protection Group, Inc. notes that the report is ...
(Date:7/24/2017)... , ... July 24, 2017 , ... ... media initiatives designed to promote awareness for Topricin’s revolutionary, natural, after-burn skin care ... one of the leading causes of long term skin conditions, including cancer. ...
Breaking Medicine News(10 mins):
(Date:7/15/2017)... , July 15, 2017 Enterin Inc., a ... to treat Parkinson,s disease (PD), today announced the completion of ... new investors New Ventures III, as well as the participation ... are absolutely thrilled to have the support of New Ventures ... our investors as validation of the potential of our platform ...
(Date:7/14/2017)... 13, 2017 It should come as no surprise ... is in the midst of a crippling opioid epidemic. According ... the number of overdose deaths from opiate-based medications has quadrupled, ... a million dead from 2001 to 2015". During this time, ... has similarly quadrupled, drawing a compelling link between prescription and ...
(Date:7/13/2017)... Calif. , July 13, 2017 RK Logistics ... Board of Pharmacy certification for its Fremont, CA ... support solutions in the Tri-Valley and San Jose ... City of Fremont , with its Fremont ... Group to provide such a powerful resource to the hundreds ...
Breaking Medicine Technology: