PRESS-NEWS.org - Press Release Distribution
PRESS RELEASES DISTRIBUTION

Myotonic dystrophy disrupts normal control of gene expression in the heart

2014-01-09
(Press-News.org) Contact information: Graciela Gutierrez
ggutierr@bcm.edu
713-798-4710
Baylor College of Medicine
Myotonic dystrophy disrupts normal control of gene expression in the heart HOUSTON – (Jan. 9, 2014) – Disruption of a transcription network controlled by MEF2 in heart tissue of people with myotonic dystrophy type 1 – an inherited form of muscular dystrophy with symptoms starting in early adulthood – affects activity of the minute bits of genetic material called microRNAs responsible for fine-tuning expression of proteins, said researchers from Baylor College of Medicine in a report that appears online in the journal Cell Reports.

Malfunction of the heart involving its ability to beat properly is the second most common cause of death in the disorder, said Dr. Thomas Cooper, professor of pathology & immunology at Baylor and corresponding author of the report.

"Our studies in both mice and human heart tissues identified many microRNAs that are affected," said Cooper.

Myotonic dystrophy occurs because parts of the gene DMPK (dystrophia myotonica-protein kinase) are duplicated abnormally, resulting in what is known as a triplet repeat. While most myotonic dystrophy research has focused on problems related to types of messenger RNAs produced from genes by altered splicing, this study demonstrates additional problems with expression of microRNAs, said Dr. Auinash Kalsotra, assistant professor of biochemistry and the first author on the study, who has now started his own research group at the University of Illinois, Urbana-Champaign.

Cooper, Kalsotra and their colleagues screened approximately 500 microRNAs and found 54 affected. A screen of human myotonic dystrophy cells identified 20 that were down regulated in the heart tissue of patients with myotonic dystrophy. MicroRNAs maintain proper gene activity so abnormal microRNA levels is likely to produce disease.

By investigating why the microRNAs were abnormal, the investigators found that the protein controlling their expression, the transcription factor MEF2, was abnormally low in myotonic dystrophy heart tissue.

MicroRNAs are fairly recently recognized genetic actors that fine tune the levels of messenger RNA, which takes the genetic code to that part of the cell where proteins are made. In this case, MEF2 affects not only the level of activity of genes but also these bits of RNA that fine-tune the proteins associated with the genes.

"This shifted the focus of research. Now a transcription factor is involved," Cooper said. Transcription factors control the expression of genes (the levels of activity). Since MEF2 controls many other genes in addition to those of microRNAs, the results identify additional abnormalities that can help explain some features of the disease.

For example, said Cooper, aberrant microRNAs result in turning on cellular factors that should not be turned on. The protein CELF1, which is upregulated in the disease, is affected by these microRNAs.

"The regulation of genes is very dynamic and finely tuned to respond to changing conditions," he said. "The abnormality that causes this disease disrupts a large network and thus has multiple effects"

Others who took part in this work include,Ravi K. Singh and Chad J. Creighton, of Baylor, and Priyatansh Gurha and Amanda J. Ward, formerly with Baylor and now with The University of Texas Health Science Center at Houston and the Isis Pharmaceuticals in Carlsbad, Calif., respectively.

### Funding for this work came from the Myotonic Dystrophy Foundation (Kalsotra), the American Heart Association (Kalsotra and Singh), the National Institutes of Health (Cooper- Grants P30CA125123, R01HL045565, R01AR060733, and R01AR045653) and the Muscular Dystrophy Association (Cooper).

Cooper holds the Donald S. Greenberg Chair of Pathology.


ELSE PRESS RELEASES FROM THIS DATE:

Mystery solved: How nerve impulse generators get where they need to go

2014-01-09
Mystery solved: How nerve impulse generators get where they need to go Study identifies essential molecule for transport of protein from neuron cell body to axon COLUMBUS, Ohio – Scientists have solved a longstanding mystery of the central nervous system, showing how a key ...

Surprising new class of 'hypervelocity stars' discovered escaping the galaxy

2014-01-09
Surprising new class of 'hypervelocity stars' discovered escaping the galaxy An international team of astronomers has discovered a surprising new class of "hypervelocity stars" – solitary stars moving fast enough to escape the gravitational grasp of ...

Study of Nepalese pilgrims challenges diagnosis of acute mountain sickness

2014-01-09
Study of Nepalese pilgrims challenges diagnosis of acute mountain sickness A study led by University of British Columbia scientists calls into question a widely used method of diagnosing acute mountain sickness. The Lake Louise Score Questionnaire has ...

A new pathway for neuron repair is discovered

2014-01-09
A new pathway for neuron repair is discovered Penn State University molecular biologists have discovered a brand-new pathway for repairing nerve cells that could have implications for faster and improved healing. The researchers describe their findings in a paper titled "Dendrite ...

Microalgae and aquatic plants can help to decrease radiopollution in the Fukushima area

2014-01-09
Microalgae and aquatic plants can help to decrease radiopollution in the Fukushima area Springer's Journal of Plant Research presents the results of a 2-year investigation in a special issue After a huge earthquake caused severe damage to the Fukushima 1 Nuclear Power ...

La Jolla Institute scientist identifies pivotal cellular protein underlying eczema

2014-01-09
La Jolla Institute scientist identifies pivotal cellular protein underlying eczema Discovery opens new therapeutic avenue for chronic skin condition affecting millions SAN DIEGO – (January 9th, 2014) Researchers from the La Jolla Institute for Allergy ...

Unfit, lean people are better protected against heart attacks than fit, obese people

2014-01-09
Unfit, lean people are better protected against heart attacks than fit, obese people In a study published in the European Heart Journal, a research team at Umeå University, Sweden, has shown that physical fitness in your teens can reduce the risk of heart ...

EU policy is driving up demand for pollination faster than honeybee numbers

2014-01-09
EU policy is driving up demand for pollination faster than honeybee numbers Research conducted by the University of Reading's Centre for Agri-Environmental Research, and funded by the EU FP7 project STEP and the Insect Pollinators Initiative Crops project, indicates that ...

A good outcome for the CHILD-INNOVAC project: successful test in humans of a nasal vaccine against pertussis

2014-01-09
A good outcome for the CHILD-INNOVAC project: successful test in humans of a nasal vaccine against pertussis The CHILD-INNOVAC European research programme, coordinated by Inserm, has enabled the development ...

Eye-catching electronics

2014-01-09
Eye-catching electronics Thin film transistors on parylene membrane This news release is available in German. Niko Münzenrieder submerges a ficus leaf in water containing pieces of a shiny metallic membrane. Using tweezers, he carefully moves one of ...

LAST 30 PRESS RELEASES:

Thirty-year mystery of dissonance in the “ringing” of black holes explained

Less intensive works best for agricultural soil

Arctic rivers project receives “national champion” designation from frontiers foundation

Computational biology paves the way for new ALS tests

Study offers new hope for babies born with opioid withdrawal syndrome

UT, Volkswagen Group of America celebrate research partnership

New Medicare program could dramatically improve affordability for cancer drugs – if patients enroll

Are ‘zombie’ skin cells harmful or helpful? The answer may be in their shapes

University of Cincinnati Cancer Center presents research at AACR 2025

Head and neck, breast, lung and survivorship studies headline Dana-Farber research at AACR Annual Meeting 2025

AACR: Researchers share promising results from MD Anderson clinical trials

New research explains why our waistlines expand in middle age

Advancements in muon detection: Taishan Antineutrino Observatory's innovative top veto tracker

Chips off the old block

Microvascular decompression combined with nerve combing for atypical trigeminal neuralgia

Cutting the complexity from digital carpentry

Lung immune cell type “quietly” controls inflammation in COVID-19

Fiscal impact of expanded Medicare coverage for GLP-1 receptor agonists to treat obesity

State and sociodemographic trends in US cigarette smoking with future projections

Young adults drive historic decline in smoking

NFCR congratulates Dr. Robert C. Bast, Jr. on receiving the AACR-Daniel D. Von Hoff Award for Outstanding Contributions to Education and Training in Cancer Research

Chimpanzee stem cells offer new insights into early embryonic development

This injected protein-like polymer helps tissues heal after a heart attack

FlexTech inaugural issue launches, pioneering interdisciplinary innovation in flexible technology

In Down syndrome mice, 40Hz light and sound improve cognition, neurogenesis, connectivity

Methyl eugenol: potential to inhibit oxidative stress, address related diseases, and its toxicological effects

A vascularized multilayer chip reveals shear stress-induced angiogenesis in diverse fluid conditions

AI helps unravel a cause of Alzheimer's disease and identify a therapeutic candidate

Coalition of Autism Scientists critiques US Department of Health and Human Services Autism Research Initiative

Structure dictates effectiveness, safety in nanomedicine

[Press-News.org] Myotonic dystrophy disrupts normal control of gene expression in the heart