Weizmann researchers find evidence that links molecular mechanism to Huntington's Disease

Weizmann researchers have found evidence that an enzyme called transglutaminase (TGase) may be the "smoking gun" behind the deadly disease Huntington's disease (HD). Their study is reported in the June 22 issue of the Proceedings of the National Academy of Sciences.

TGase occurs naturally throughout the body, and is catalyst for processes associated with healing, such as skin formation and wound healing. But a 1993 study by Prof. Howard Green (now of the Department of Cell Biology, Harvard Medical School, Boston) suggested that because of their polymerizing properties, they might also act as a catalyst for aggregating a protein associated with HD called Huntingtin (htt). Prof. Lawrence Steinman (then of the Weizmann Institute's Department of Immunology, now of Stanford University's Department of Neurological Science), together with Weizmann graduate student, Marcela Karpuj and a team of researchers in the United States, have now provided clear evidence of elevated TGase activity in the postmortem brains of HD patients.

Patients who suffer from HD have aggregations "visible clumps"of htt in their brains, but not in other tissues. This makes aggregated htt a good pathological indicator for the disease. Steinman, Karpuj, and the other members of the team decided to look for a correlation between clumps of htt and TGase among patients with HD. They found elevated TGase activity in the cortex, the cerebellum, and the brain nuclei--all areas in the brain where htt gets concentrated into nuclear inclusions. Correspondingly, they found reduced TGase activity in lymphoblastoid cells--areas where htt does not aggregate. TGase, therefore, appears to be the "smoking gun" that leads to the formation of nuclear inclusions (htt aggregations) in HD patients.

Their research is unique, because for the first time, researchers were able to report on TGase activity in the brains of HD patients rather than in guinea pigs or rats. They compared five postmortem brains of HD

Contact: Ellen Shnidman
American Committee for the Weizmann Institute of Science

Page: 1 2 3

Related biology news :

1. Weizmann scientists invention moves forward: Cancer diagnosis technique gets FDA clearance
2. Psoriasis treatment based on Weizmann Institute Research passes phase II clinical trials
3. Weizmann Institute scientists report why taste and smell differ among individuals
4. Weizmann Institute scienists find that stem cells in the bone marrow become liver cells
5. Weizmann institute scientists solve the 3-D structure of the enzyme involved in Gaucher disease
6. Prize to Weizmann Institute professor for landmark work in stem cell transplantation
7. New witchweed-fighting method, presented by CIMMYT and Weizmann Institute scientist
8. Weizmann Institute scientists win global competition in predicting protein-protein interactions
9. Vaccination following spinal cord injury: Innovative Weizmann Institute approach limits paralysis
10. Weizmann Institute scientists block loss of eyesight in animals with a glaucoma-like disease
11. Weizmann Institute researcher named to Israel Academy Of Sciences And Humanities

Post Your Comments:

(Date:1/30/2019)... (PRWEB) , ... January 30, ... ... organization that identifies, counsels, and supports high-growth, high-impact life science and biotechnology ... representing 10 national nonprofit disease foundations and disease-focused venture funds to discuss ...
(Date:1/21/2019)... ... January 20, 2019 , ... Energetiq Technology, a world leader in high brightness ... SPIE BIOS and SPIE Photonics West events taking place in San Francisco on February ... at Bios and in booth #941 at Photonics West. There will be live demonstrations ...
(Date:1/20/2019)... , ... January 18, 2019 , ... Boston-based Laboratory as ... in an effort to better express its continued expansion and service offerings for ... for a new kind of lab: one that is more agile and adaptable than ...
Breaking Biology News(10 mins):
(Date:1/28/2019)... ... January 28, 2019 , ... Fragment-based lead discovery (FBLD) ... this early stage, false positives can be problematic due to high levels of ... screening techniques are highly sensitive to detect weak interactions. Nanomed , leading ...
(Date:1/24/2019)... POWAY, Calif. (PRWEB) , ... January 22, 2019 , ... ... been using the Pall Veterinary Platelet Enhancement Therapy System (V-PET™) for nearly ... perform VetStem Regenerative Cell Therapy since 2007 and began using V-PET™ 2016. ...
(Date:1/24/2019)... ... January 23, 2019 , ... Through its ... research support in study design, planning, sample collection, and analysis to researchers at ... Campus in Trinidad led by Dr. Elaine Monica Davis, MBBS, MPhil, Lecturer of ...
(Date:1/20/2019)... ... 2019 , ... On January 15 of this year, the Parents’ Guide to ... director James L. Sherley, M.D., Ph.D. The PGCB was founded by former physicist ... Now in its 21st year of operation, the PGCB continues its mission to ...
Breaking Biology Technology:
Cached News: