Navigation Links
Study Reveals Protein Behind RDEB’s Painful Symptoms and How Stem Cells Might Stop It

A study recently published in STEM CELLS suggests unrestricted somatic stem cells (USSCs) derived from human cord blood can ease fibrosis and inflammation in a painful type of skin disorder called recessive dystrophic epidermolysis bullosa (RDEB). The stem cells also can potentially prevent malignant tumors, a frequent side effect of RDEB, from forming.

An estimated 25,000 people in the United States alone live with some form of RDEB, a genetic condition characterized by extremely fragile skin. Patients can experience painful blisters and skin erosions from something as simple as rubbing or scratching. Nearly every patient with a severe generalized form of RDEB has “mitten” deformities of the hands and feet. Even after surgical intervention, the deformities frequently reoccur. In the most severe cases, internal organs are affected and vision loss, disfigurement and other serious medical problems can occur.

Patients with RDEB are also at high risk for developing cutaneous squamous cell carcinoma, an aggressive type of skin cancer. Currently there is no treatment for RDEB.

In the study in STEM CELLS, New York Medical College colleagues Mitchell Cairo, M.D., and Yanling Liao, Ph.D., led a team intent on uncovering the role of a protein called transforming growth factor beta (TGFß) in RDEB. The TGFß signaling pathway is involved in many cellular processes in embryos and adults, including cell growth, differentiation, apoptosis and more. As such, TGFß1 plays an essential part in wound healing. However, recent studies have also demonstrated that TGFß signaling is associated with fibrosis and malignancy in RDEB animal models (col7a1-/- mice) and humans.

Along with investigating the dynamics of TGFß signaling and RDEB, Drs. Cairo and Liao wanted to take the next step in their previous study, also published in STEM CELLS, in which they showed how an injection of USSCs significantly improved wound healing and extended the life of col7a1-/- mice. This time they wanted to learn what happened when the injections were given earlier and repeatedly.

They began by injecting one group of col7a1-/- mice with a dose of USSCs. A second group of col7a1-/- mice were administered a buffer (dextran/human serum albumin, or D/HSA). As a control, they did the same using wild type mice. The injections were administered to each animal starting at birth, and then once a week for a total of four weeks.

“We saw the activation of TGFß signaling in the col7a1-/- mice as early as a week after birth, starting in the interdigital folds of the paws – similar to the ‘mitten’ effect you see in human patients. This was accompanied by increased deposits of collagen fibrils as well as elevated levels of MMP-9 and MMP-13, which are malignancy biomarkers,” Dr. Cairo said.

“But interestingly,” Dr. Liao added, “when we compared the group of col7a1-/- mice injected with USSCs to the col7a1-/- mice given the buffer, we found that the USSCs had suppressed TGFß signaling and MMP-9 and MMP-13 expression, while upregulating TGFß3 and decorin, which reduces fibrosis and scarring.”

“This suggests to us that an early medical intervention using USSCs before full activation of TGFß signaling may be ideal for preventing mitten deformity in patients with RDEB, and for mitigating epithelial malignancy, in addition to their anti-inflammatory and antifibrotic functions.”

Dr. Nolta, Editor in Chief of Stem Cells, commented, “this study is very important for determining the stage at which RDEB could potentially be treated through cell therapy, in order to minimize the scarring and damage that occurs in the patients’ skin. We are proud to publish this important work suggesting ways to better understand and treat this devastating disease.”


The full article, “Cord Blood Derived Stem Cells Suppress Fibrosis and May Prevent Malignant Progression in Recessive Dystrophic Epidermolysis Bullosa,” can be accessed at

About the Journal: STEM CELLS, a peer reviewed journal published monthly, provides a forum for prompt publication of original investigative papers and concise reviews. The journal covers all aspects of stem cells: embryonic stem cells/induced pluripotent stem cells; tissue-specific stem cells; cancer stem cells; the stem cell niche; stem cell epigenetics, genomics and proteomics; and translational and clinical research. STEM CELLS is co-published by AlphaMed Press and Wiley.

About AlphaMed Press: Established in 1983, AlphaMed Press with offices in Durham, NC, San Francisco, CA, and Belfast, Northern Ireland, publishes three internationally renowned peer-reviewed journals with globally recognized editorial boards dedicated to advancing knowledge and education in their focused disciplines. STEM CELLS® ( is the world's first journal devoted to this fast paced field of research. THE ONCOLOGIST® ( is devoted to community and hospital-based oncologists and physicians entrusted with cancer patient care. STEM CELLS TRANSLATIONAL MEDICINE® ( is dedicated to significantly advancing the clinical utilization of stem cell molecular and cellular biology. By bridging stem cell research and clinical trials, SCTM will help move applications of these critical investigations closer to accepted best practices.

About Wiley: Wiley, a global company, helps people and organizations develop the skills and knowledge they need to succeed. Our online scientific, technical, medical and scholarly journals, combined with our digital learning, assessment and certification solutions, help universities, learned societies, businesses, governments and individuals increase the academic and professional impact of their work. For more than 200 years, we have delivered consistent performance to our stakeholders. The company's website can be accessed at

Read the full story at

Source: PRWeb
Copyright©2018 Vocus, Inc.
All rights reserved

Related medicine news :

1. New study assesses injuries seen in the emergency department to children of teenage parents
2. Study finds that Americans want doctors guidance on genetic test results
3. FDA awards $2.25M grant to study immunosuppresive drug in high-risk patients
4. Study using stem cells to improve organ transplantation to receive $12 million
5. TGen-led study reveals TWEAK-Fn14 as key drug target
6. In animal study, cold turkey withdrawal from drugs triggers mental decline
7. Journal of Traumatic Stress Study Analyzes Psychological Effects of Marathon Bombing on Boston-Area Veterans with Post-Traumatic Stress Disorder (PTSD)
8. Investigational Drug Effective in Treating Iron Deficiency in Chronic Kidney Disease Patients on Dialysis: Study
9. Searchen Releases Statement on Celebrity and Athletic Endorsement Study
10. Hope for transplant patients as study finds key to organ scarring
11. Princeton study: Military children and their families remain an invisible subculture
Post Your Comments:
(Date:4/18/2019)... ROOT, Switzerland (PRWEB) , ... April 18, 2019 , ... ... market. The SOULMATE range is designed to support the bond between human and animal ... unique and formulated by certified Swiss herbal specialists with decades of experience. They are ...
(Date:4/18/2019)... (PRWEB) , ... April 18, 2019 , ... She went ... a pair of eyeglasses, said her mother, Cheryl Reilly. Those “goofy tests,” performed by ... that Mikayla Reilly, then 12 years old, had such narrow range of visual awareness, ...
(Date:4/17/2019)... ... 17, 2019 , ... With Fentanyl overdose deaths continuing to claim lives and ... toll by looking for an alternative to naloxone. Researchers are presenting their findings ... , Naloxone (often known by the name brand Narcan) has been utilized increasingly since ...
(Date:4/17/2019)... ... 17, 2019 , ... The IBS Patient Support Group, an ... designate April 19th as World IBS Day to raise awareness for Irritable Bowel ... their pursuit to find treatments. Irritable Bowel Syndrome is a common digestive disorder ...
(Date:4/17/2019)... ... April 17, 2019 , ... An upcoming ... diagnostic technologies. This episode is scheduled to air 3Q 2019 on FOX Business. ... focus on Credo Biomedical Pte. Ltd. (Credo Biomedical), a molecular diagnostic company from ...
Breaking Medicine News(10 mins):
(Date:4/17/2019)... ... April 17, 2019 , ... Lillibridge ... of the nation’s largest owners and operators of medical office buildings (MOBs), will ... experts during the Building Owners and Managers Association (BOMA) International Medical Office Buildings ...
(Date:4/17/2019)... ... ... Clinical trials of new cancer treatments have historically focused on disease type by tumor ... oncology and genomic characterization of tumors, we are now able to test new therapies ... ability has created a paradigm shift in oncology trials. , In basket trials, cancers ...
(Date:4/17/2019)... ... April 17, 2019 , ... Women’s Excellence in Obstetrics and Gynecology ... a health concern, you want to be seen by a provider. We believe ... to their normal routine as soon as possible.” Dr. Jonathan Zaidan, MD, FACOG, President ...
Breaking Medicine Technology: