Navigation Links
Mechanism for neurodenegerative diseases linked to transport proteins

Hampering the transport of proteins within cells may underlie several adult-onset neurodegenerative diseases, such as Huntington's, ALS and Kennedy disease. Understanding how this cell transport is blocked in these diseases may offer targets for future therapy.

In a new study published online June 4 in Nature Neuroscience, researchers from the University of Illinois at Chicago College of Medicine showed how a chemical pathway that is obstructed in Kennedy disease interferes with a cellular distribution system called "fast axonal transport" that moves proteins from where they are synthesized to where they are needed in the cell.

This transport system is critical in neurons because these cells can be as much as three feet long, says Dr. Scott Brady, professor and head of anatomy and cell biology at UIC.

"A breakdown in fast axonal transport would selectively kill neurons because neurons are especially dependent on the transport system," Brady said.

Kennedy disease is also known as spinal and bulbar muscular atrophy, or SBMA. Like the better-known ALS and Huntington's, it is a rare but devastating disease, affecting one in 40,000 people, usually between the ages of 30 and 50. Huntington's strikes about four times as many.

Neurodegenerative diseases like SBMA are caused by the lengthening of part of a gene that encodes repetitions of the amino acid glutamine in the protein. Although different genes are affected, all of the polyglutamine-expansion or "polyQ" diseases are characterized by symptoms that begin in middle age and by the loss of certain types of neurons through a pattern in which the neuron's terminals die before the cell body. PolyQ genes are expressed in many types of cells, but only neurons are affected.

Earlier studies had linked specific neurodegenerative diseases to mutations in proteins involved in intracellular transport. This led researchers to wonder if the deranged polyQ proteins inhibit fast axonal tr ansport in several diseases, including SBMA, in which a mutation in the receptor for testosterone leads to the loss of motor neurons. In the new study, Brady and his co-workers were able to show that polyQ-AR, the mutated protein in SBMA, caused inhibition of fast axon transport by activating an enzyme called JNK that can inhibit these transport proteins.

Brady said this is the first proposed mechanism for polyQ diseases that explains why only nerve cells die and why the terminals die before the cell body. The link to the activation of the JNK enzyme suggests a new therapeutic target that might limit, delay or perhaps prevent progressive neurodegeneration, the researchers conclude.


'"/>

Source:University of Illinois at Chicago


Related biology news :

1. Mechanism for the captation of nutrients in plants- unknown to date
2. Mechanism regulating tooth shape formulation found
3. Mechanism for Epstein-Barr virus proteins role in blood cancers discovered
4. Mechanism for memory revealed in neurons of electric fish
5. Mechanism identified for promising neurological drug
6. Mechanisms involved with tumor relapse identified
7. Mechanism of nicotines learning effects explored
8. Ecological destruction fuels emerging diseases
9. Molecular machine may lead to new drugs to combat human diseases
10. Female sex hormones play a vital role in defense against sexually transmitted diseases
11. First atlas of key brain genes could speed research on cancer, neurological diseases
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:3/3/2016)... 2016  FlexTech, a SEMI Strategic Association Partner, awarded ... & Development, Leadership in Education, and, in a category ... th year of the FLEXI Awards and the ... from past years . Judging was done on ... of criteria, by a panel of non-affiliated, independent, industry ...
(Date:3/2/2016)... DUBLIN , March 2, 2016 /PRNewswire/ ... has announced the addition of the  "Global ... to their offering.  ,     (Logo: http://photos.prnewswire.com/prnh/20130307/600769) , ... sector to grow at a CAGR of ... http://www.researchandmarkets.com/research/wzwqtz/global_biometrics ) has announced the addition ...
(Date:3/1/2016)... , March 1, 2016  (RSAC Booth #3041) – ... a whopping $118 billion is lost to false positives, ... and inaccurate fraud detection. At the RSA Conference 2016, ... way companies handle authentication by devaluing the data fraudsters ... analytics. --> --> ...
Breaking Biology News(10 mins):
(Date:4/29/2016)... April 29, 2016 According ... Market Research "Separation Systems for Commercial Biotechnology Market ... and Forecast 2015 - 2023", the separation systems ... 10,665.5 Mn in 2014 and is projected to ... to 2023 to reach US$ 19,227.8 Mn in ...
(Date:4/28/2016)... India , April 28, 2016 ... JT, Stirling, and Brayton Cryocoolers), Service (Technical Support, Product ... and Geography - Global Forecast to 2022", published by ... USD 2.94 Billion by 2022, at a CAGR of ... 70 market data Tables and 94 Figures spread through ...
(Date:4/28/2016)... ... 2016 , ... Morris Midwest ( http://www.morrismidwest.com ), a division ... its Maple Grove, Minnesota technical center, May 11-12. The event will feature ... 20 leading suppliers of tooling, accessories, software and other related technology will participate ...
(Date:4/27/2016)... , April 27, 2016 ... NSK) (OTCPink: NSKQB) ( Frankfurt : ... ihre Pressemitteilung vom 13. August 2015 die Genehmigung ... Finanzen um zusätzliche 200.000.000 Einheiten auf 400.000.000 Einheiten ... zu bringen. Davon wurden 157.900.000 Einheiten mit dem ...
Breaking Biology Technology: