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:10/13/2017)... ... October 13, 2017 , ... Ellevate Network, the leading network for professional ... action towards gender equality at their inaugural Summit in New York City in June. ... a social audience of over 3 million. To watch the Mobilize Women video, ...
(Date:10/13/2017)... ... , ... “The Journey: From the Mountains to the Mission Field”: the story ... the Philippines. “The Journey: From the Mountains to the Mission Field” is the creation ... has taught all ages and currently teaches a class of ladies at her church, ...
(Date:10/12/2017)... Wis. (PRWEB) , ... October 12, 2017 , ... ... standard products to meet the demand of today’s consumer and regulatory authorities worldwide. ... of probiotic experts and tested to meet the highest standard. , These ...
(Date:10/12/2017)... ... October 12, 2017 , ... Dr. Parsa Mohebi, the ... medical article to the newly revamped Cosmetic Town journal section, featuring ... transplant procedure known as Follicular Unit Extraction (FUE). , Dr. Mohebi ...
(Date:10/12/2017)... ... October 12, 2017 , ... ... first interactive health literacy software tool, and the Cancer Patient Education Network (CPEN), ... patient education, today announce a new strategic alliance. , As CPEN’s strategic ...
Breaking Medicine News(10 mins):
(Date:10/7/2017)... 2017   Provista, a proven leader in ... in purchasing power, today announced a new resource area ... Newsroom is the online home for case studies, ... bios, news releases, slideshows and events. ... of resources at their fingertips, viewers can also watch ...
(Date:10/4/2017)... Mass. , Oct. 4, 2017  According to the Centers for ... the end of October . PhysicianOne Urgent Care is helping communities across ... Westchester, NY , by offering no-cost* flu shots through the ... as mandated by certain health insurance regulations. ... The best time to get a flu shot is by the ...
(Date:10/2/2017)... , Oct. 2, 2017  Eli Lilly and Company ... results for the third quarter of 2017 on Tuesday, ... call on that day with the investment community and ... The conference call will begin at 9 a.m. ... access a live webcast of the conference call through ...
Breaking Medicine Technology: