Navigation Links
Researchers discover genetic cause for word-finding disease

Northwestern University researchers have discovered a genetic cause of a mysterious neurological disease in which people have trouble recalling and using words. The illness, Primary Progressive Aphasia, differs from Alzheimer's Disease in which a person's memory becomes impaired. In PPA, a little known form of dementia, people lose the ability to express themselves and understand speech.

"This discovery, for the first time, provides a molecular approach to understanding the causes and eventually the treatment for this disease, " said Marsel Mesulam, M.D., lead author of the study and the Ruth and Evelyn Dunbar Professor of Neurology and Psychiatry at The Feinberg School of Medicine at Northwestern University. Mesulam was the first scientist to identify the word-finding disease in 1982. He estimated it affects tens of thousands of people, though no exact statistics are available. People can begin to show symptoms of PPA as early as in their 40's and 50's.

Scientists discovered the gene mutation, called a progranulin gene mutation, in two unrelated families in which nearly all the siblings suffered from PPA. In the first family, three out of four siblings had the disease; in the second family, two out of three had it. These particular mutations were not observed in the healthy siblings or in more than 200 control samples.

The study was published in January Archives of Neurology and is discussed in an editorial in the journal.

"We're dealing with one of the most puzzling phenomenons in neurology," said Mesulam, director of Northwestern's Cognitive Neurology and Alzheimer's Disease Center. "Here is a disease that specifically attacks the language part of the brain on the left side. What makes it so specific? How does the disease target the language areas?"

"This finding will help us explore not just what causes the disease but the uniquely identifying features of human language. That's a pretty big question," added Mes ulam.

MRI imaging of the brains of people with PPA shows the language part in the perisylvian region of the left hemisphere has shrunk. By comparison, Alzheimer's Disease targets the hippocampus on both sides of the brain.

One of the oddities about PPA is that even when people have lost their ability to speak, they are still able to maintain their hobbies and perform other tasks. "One of my patients redid his vacation home and rebuilt all the cabinets himself. Another took up sculpturing and one kept up her organic garden. We have patients who do very complicated things even when they can't put two sentences together," Mesulam said. Alzheimer's patients lose interest in their hobbies, family life and just sit doing nothing, he noted.

As PPA progresses over 10 to 15 years, however, patients eventually lose their ability to function independently.


'"/>

Source:Northwestern University


Related biology news :

1. Researchers discover way to make cells in the eye sensitive to light
2. Researchers find how protein allows insects to detect and respond to pheromones
3. Researchers Uncover Key Step In Manufacture of Memory Protein
4. Researchers reveal the infectious impact of salmon farms on wild salmon
5. Researchers identify target for cancer drugs
6. Researchers discover molecule that causes secondary stroke
7. Researchers find missing genes of ancient organism
8. Researchers trace evolution to relatively simple genetic changes
9. Researchers add new tool to tumor-treatment arsenal
10. UF Researchers Map Bacterial Proteins That Cause Tooth Loss
11. VCU Researchers Identify Networks Of Genes Responding To Alcohol In The Brain
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:8/23/2017)...  The general public,s help is being enlisted in what,s thought to ... and on the human body –and are believed to affect health.  ... The Microbiome Immunity Project is the largest ... the gut. The project's goal is to help advance scientific knowledge of ... ...
(Date:6/30/2017)... 2017 Today, American Trucking Associations announced ... face and eye tracking software, became the newest ... "Artificial intelligence and advanced sensing ... a driver,s attentiveness levels while on the road.  ... detect fatigue and prevent potential accidents, which could ...
(Date:5/23/2017)... the first robotic gym for the rehabilitation and functional motor sense evaluation ... Genoa, Italy . The first 30 robots will be available from ... . The technology was developed and patented at the IIT laboratories ... Technology thanks to a 10 million euro investment from entrepreneur Sergio Dompè. ... ...
Breaking Biology News(10 mins):
(Date:10/11/2017)... ... 11, 2017 , ... At its national board meeting in ... Sheikh, the co-founder, CEO and chief research scientist of Minnesota-based Advanced Space Technology ... in ARCS Alumni Hall of Fame . ASTER Labs is a technology ...
(Date:10/11/2017)... ... 2017 , ... ComplianceOnline’s Medical Device Summit is back for its 4th year. ... San Francisco, CA. The Summit brings together current and former FDA office bearers, regulators, ... government officials from around the world to address key issues in device compliance, quality ...
(Date:10/11/2017)... LAGUNA HILLS, Calif. , Oct. 11, 2017 /PRNewswire/ ... London (ICR) and University of ... tool to risk-stratify patients with multiple myeloma (MM), in a ... . The University of Leeds is ... Myeloma UK, and ICR will perform the testing services to ...
(Date:10/11/2017)... (PRWEB) , ... October 11, 2017 , ... ... Administration (FDA) has granted orphan drug designation to SBT-100, its novel anti-STAT3 (Signal ... the treatment of osteosarcoma. SBT-100 is able to cross the cell membrane and ...
Breaking Biology Technology: