Navigation Links
Iron-moving malfunction may underlie neurodegenerative diseases, aging
Date:9/22/2008

't found, and we thought there must be an unidentified iron transporter protein, possibly TRPML1."

Unfortunately, TRPML1 isn't the easiest protein to study. Instead of residing in the cell's easily-accessed outer membrane, where many other proteins nestle, it hides in a tiny, interior pocket called lysosome. To probe the protein, Xu's group had to modify a technique known as the patch clamp, in which a micropipette and electrodes are attached to a cell membrane to record the activity of individual or multiple proteins that serve as channels for charged particles (ions) moving in and out of cells. With their modification, which they call the lysosome patch clamp, Xu's group was able to record TRPML1 activity in the tiny lysosome.

They found that TRPML1 was indeed capable of ferrying iron out of the lysosome. But was there any evidence that interfering with that ability might result in ML4 symptoms? To address that question, Xu's group studied defective TRPML1 proteins bearing the same mutations as those found in ML4 patients. Mutations associated with severe symptoms were the least adept at shuttling iron, while those associated with milder symptoms were more proficient, although still not fully functional.

Further experiments confirmed that when TRPML1 is defective, iron becomes trapped in the lysosome. One result of the buildup is formation of a brownish waste material, lipofuscin, known as the "aging pigment." In skin cells, lipofuscin is the culprit responsible for the dreaded liver spots that appear with increasing age, but in nerve, muscle and other cells, its accumulation has more serious consequences.

"How lipofuscin causes problems in neurons and muscles is not clear, but it's believed that this is garbage that, in time, compromises the normal function of the lysosome," Xu said. "And we know the lysosome is important for all kinds of cell biology, particularly the recycling of intracellular components, so if it's damaged,
'/>"/>

Contact: Nancy Ross-Flanigan
rossflan@umich.edu
734-647-1853
University of Michigan
Source:Eurekalert

Page: 1 2 3

Related biology news :

1. Complex dynamics underlie bark beetle eruptions
2. Antioxidant overload may underlie a heritable human disease
3. Abnormal fat metabolism underlies heart problems in diabetic patients
4. Gene therapy slows progression of fatal neurodegenerative disease in children
5. The accumulation of sugar in neurons may explain the origin of several neurodegenerative diseases
6. Researchers identify proteins involved in new neurodegenerative syndrome
7. Certain diseases, birth defects may be linked to failure of protein recycling system
8. New chimeric mouse model for human liver diseases, drug testing
9. Syntermed licenses Emory ERTb software for enhanced cardiac imaging
10. Old before their time? Aging in flies under natural vs. laboratory conditions
11. Scientists use remote satellite imaging to predict outbreaks of infectious disease
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:8/20/2014)... bags could one day be made out of cocoa, ... now reporting. The novel process they developed and their ... agricultural and plastic waste problems, appear in the ACS ... Bayer and colleagues at the Italian Institute of Technology ... 2012, its production reached 288 million tons worldwide, but ...
(Date:8/20/2014)... When a colony of honeybees grows to about 4,000 ... reproductive cycle: the building of a special type of ... team of experts from the Department of Neurobiology and ... what starts the reproductive cycle of honeybee colonies. The ... The Science of Nature . , Reproduction isn,t ...
(Date:8/20/2014)... POINT ROBERTS, Washington , August 20, 2014 ... Addressing Security Concerns in the Payment Industry?   ... leading sectors including biometrics, issues commentary from analysts and experts ... Peaslee , Managing Partner at Thrive Analytics, Isabelle Moeller ... CEO of NXT-ID, Inc. (OTCBB: NXTD) ( OTCQB:NXTD ), a ...
Breaking Biology News(10 mins):Worker bees 'know' when to invest in their reproductive future 2Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 2Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 3Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 4Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 5Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 6Digital Wallets for a Digital World - the Players and the Concerns for Consumer Adoption 7
... the Institute for Stem Cell Biology and Medicine have demonstrated ... be genetically manipulated and coaxed to develop into mature T-cells, ... The study, to be published the week of July 3 ... Academy of Sciences, found that it is possible to convert ...
... skyrocketed from 497 cases reported in 1982 (the first year ... , Lyme disease, like other zoonoses, is ... a blood meal from a vertebrate host. In the eastern ... blacklegged ticks, Ixodes scapularis, which feed on a wide variety ...
... University of North Carolina at Chapel Hill has identified ... potentially dangerous interactions with certain medications. , For almost ... and pharmacists to avoid grapefruit juice if they are ... control blood pressure or lower cholesterol. Studies have shown ...
Cached Biology News:UCLA researchers develop T-cells from human embryonic stem cells 2A new view on Lyme disease: Rodents hold the key to annual risk 2A new view on Lyme disease: Rodents hold the key to annual risk 3Study identifies substances in grapefruit juice that interact dangerously with some drugs 2
(Date:8/20/2014)... The 6th Annual Arrowhead Personalized and ... 29-30 in San Francisco, CA. This year’s conference brings ... and clinicians focused on addressing and solving the significant ... twelve years since the sequencing of the human genome, ... pharmacogenetic information in the label and molecular diagnostics have ...
(Date:8/20/2014)... your ear could improve the health of your heart, ... of Leeds used a standard TENS machine like those ... to the tragus, the small raised flap at the ... ear canal. , The stimulation changed the influence of ... nervous signals that can drive failing hearts too hard. ...
(Date:8/19/2014)... 20, 2014 One of the ... is an inadequate vascular supply. Nutrient and gas ... tissues is critical for successful regeneration and integration ... mitigate deficiencies in vascularization and promote angiogenesis in ... optimizing scaffold design and architecture, and enhancing scaffolds ...
(Date:8/19/2014)... Pa. , Aug. 19, 2014 A ... has been nominated for a Centers for Disease Control ... addressing a major public health concern. ... System, is one of a team of researchers nominated ... Charles C. Shepard Science Award for Data Methods and ...
Breaking Biology Technology:6th Annual Personalized and Precision Medicine Conference Bringing Together Thought Leaders to Discuss Clinical Implementation, Dx Reimbursement, Big Data, and Sequencing 2'Tickling' your ear could be good for your heart 2Vascularization in Tissue Engineering Highlighted at 12th New Jersey Symposium on Biomaterials Science 2Geisinger investigator nominated for federal award 2Geisinger investigator nominated for federal award 3
... ZymoGenetics, Inc. (Nasdaq:,ZGEN) announced that the company ... update on the regulatory review of recombinant thrombin,(rThrombin). ... on August 22 at 5:00,p.m. Eastern Time by ... the call approximately 10 minutes prior to,the scheduled ...
... Missourians Introduce New Ballot Initiative to Pursue Cures for Patients, ... without involving controversial Human Cloning, WASHINGTON, Aug. ... Missouri to ensure that taxpayer funds,support cures for patients, without ... Research Council, issued the,following statement on the new initiative, Cures ...
... The U.S. Food and Drug,Administration (FDA) today approved ... ages 13-17 and for the short-term,treatment of bipolar ... I disorder in children and adolescents ages 10-17. ... more than 430 adolescents,ages 13-17, in the treatment ...
Cached Biology Technology:ZymoGenetics Will Hold Conference Call Today at 5:00 p.m. Eastern Time to Provide Update on rThrombin 2FDA Approves RISPERDAL(R) to Treat Adolescents with Schizophrenia and Children and Adolescents with Bipolar Mania 2FDA Approves RISPERDAL(R) to Treat Adolescents with Schizophrenia and Children and Adolescents with Bipolar Mania 3
TEMPLIPHI HT SEQ AMPL KIT, 1 EA. Category: Sequencing Template Amplification....
This Malachite Green Phosphate Detection Kit...
Request Info...
1+ mg expected yield of purified protein, expressed in insect cells - excludes cDNA...
Biology Products: