Navigation Links
Bone marrow-derived stem cells may offer novel therapeutic option for skin disorder
Date:12/4/2008

(WASHINGTON, December 3, 2008) Stem cells derived from bone marrow may serve as a novel therapeutic option to treat a disease called epidermolysis bullosa (EB), a disorder characterized by extraordinarily fragile skin, according to a study prepublished online in Blood, the official journal of the American Society of Hematology.

Epidermolysis bullosa is a disorder characterized by extraordinarily fragile skin and blistering on touch, akin to third degree burns. While the disease is often lethal in the neonatal period, more severe forms of the disease, such as recessive dystrophic EB (referred to as RDEB), can lead to years of painful blistering and mutilating scarring. The condition is caused by significantly reduced collagen type 7 protein (col7) production, a key component of the anchoring fibrils that connect the cutaneous membranes to the dermis of the skin and mucosal tissues in the gastrointestinal tract. A lack of these fibrils means the dermal-epidermal connection is very sensitive, and any action, which can include simple functions such as walking or eating, and the touch of clothing, creates friction between the skin layers that creates blisters and painful sores.

Children with RDEB, who are often referred to as butterfly children because their skin is said to be as sensitive as butterfly wings, develop painful skin and mucosal blistering, mutilating scarring, alopecia (hair loss), and other erosions shortly after birth. As a result of the extreme fragility of the skin and the chronic trauma of friction, RDEB patients often develop squamous cell carcinomas (a form of skin cancer). There is currently no cure for the disease, and palliative care includes complex bandaging, surgical removal of damaged tissue, and nutritional support.

We have been looking into stem cells as viable treatment options for correction of conditions such as epidermolysis bullosa, because they can produce extracellular matrix proteins, said Jakub Tolar, MD, PhD, of the University of Minnesota and lead author of the study. In this condition, the skin, the largest organ in the body, can significantly benefit from a renewable source of healthy cells that can help improve the connection between the dermis and epidermis and strengthen the skin against everyday stresses.

In this study, researchers worked with a mouse model of RDEB-infused bone marrow cells to determine if they would increase production of the col7 protein and formation of anchoring fibrils, and improve survival in the mouse recipients. The research team used bone marrow cells enriched for hematopoietic (stem cells that can develop into most blood cell types) and progenitor cells to increase the concentration of cells with the capacity to produce col7. The team tested these cells against non-enriched stem cells to determine their benefit to the treated mice.

Results of the study found that when injected into mice with RDEB, these specially selected marrow-derived stem cells diminished the disease process. They traveled to the diseased skin areas, increased protein and anchoring fibrils, prevented blister formation and extended survival. In contrast to other marrow cells, the selected cells extended the median survival time versus untreated or non-enriched marrow-treated recipients (10.0 versus 5.6 versus 6.0 days, respectively). Three of the 20 mice treated with the enriched cells benefited enough from the treatment to survive longer than the treatment period (untreated RDEB mice usually die within two weeks). Importantly, each survivor demonstrated marked improvement of new blister formation (blisters develop consistently in the areas of trauma, including footpads due to walking or in the oral cavity due to eating) with some evidence of old blisters healing.

Our data provide the first evidence that a selected population of marrow cells can connect the epidermis and dermis in a mouse model of the disease and offer a potentially valuable approach for treatment of human RDEB and other extracellular matrix disorders. These results provide proof of principle of bone marrow transfer to repair the basement membrane defect in RDEB, and they warrant a clinical trial to assess the safety and efficacy of treatment of human RDEB by means of hematopoietic cell transplantation, said Dr. Tolar.

Research suggests that the systemic infusion of wild-type bone marrow cells could provide benefit to other human disorders of the extracellular matrix. Efforts are underway to identify the requirements of bone marrow-derived stem cells capable of efficiently homing to wounded skin and producing an array of extracellular matrix proteins. As the principal advantage of systemic therapy is its potential to target not only the skin but also the mucosa of the mouth and gastrointestinal tract, the clinical testing of efficacy of human bone marrow for the treatment of human RDEB is underway to determine whether it is of more substantial benefit than local protein, gene, or cellular therapies currently being investigated by other researchers.

An estimated 50 in 1 million live births are diagnosed with EB. The disorder occurs in every racial and ethnic group throughout the world and affects both sexes.


'/>"/>

Contact: Patrick C. Irelan
pirelan@hematology.org
202-292-0253
American Society of Hematology
Source:Eurekalert

Related medicine news :

1. Tumors use enzyme to recruit regulatory T-cells and suppress immune response
2. Brain cells work differently than previously thought
3. Embryonic Stem Cells Repair Human Heart
4. Embryonic Human Stem Cells May Help Repair Heart Muscle, Lab Study Shows
5. Circulating fats kill transplanted pancreas cells, study shows
6. Experimental anti-cancer drug made from corn lillies kills brain tumor stem cells
7. Melanoma drug revs immune cells but cancer cells ignore it
8. Scientists explain how insulin secreting cells maintain their glucose sensitivity
9. Embryonic stem cells used to grow cartilage
10. Molecular probe paints cancer cells in living animals, Stanford researchers find
11. USC researcher identifies stem cells in tendons that regenerate tissue in animal model
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:3/24/2017)... ... March 24, 2017 , ... Judy Buchanan, co-owner ... in Frederick, MD. Judy says, “I am passionate about sharing Reiki as a ... very difficult and challenging time.” , A Certified Medical Reiki™ Master trained by ...
(Date:3/24/2017)... ... ... “Vintage and Harvest A Cultivation of Christian Love” is the creation of published author, ... his wife, Anna Marie. He and his wife are the proud parents of four ... of “Shadow and Substance.” , “Love, the agape kind, is seen as more than ...
(Date:3/23/2017)... & Seattle, WA (PRWEB) , ... March 23, 2017 , ... ... health emergency and now estimates that there could be four million Zika-related cases in ... epidemics to date with numbers of US cases reported per year skyrocketing to an ...
(Date:3/23/2017)... Alexandria, Virginia (PRWEB) , ... March 23, 2017 ... ... quality public health services to their communities, 16 more public health departments have ... This week’s decisions bring another 4.5 million people into the expanding network of ...
(Date:3/23/2017)... ... 2017 , ... The IoT (Internet of Things) is revolutionizing the way the ... on businesses and individual consumers alike. Laboratories can maximize their profit margin by ... from $4 trillion to $11 trillion dollars by the year 2025. McKinsey expects the ...
Breaking Medicine News(10 mins):
(Date:3/24/2017)... , March 24, 2017 Abdominal Aortic Aneurysm Repair Devices ... by 2022, Globally, registering a CAGR of 5.1% from 2016 to 2022. The endovascular ... to dominate the market during the study period. Continue ... ... ...
(Date:3/24/2017)... Mar. 24, 2017 Research and Markets ... the U.S.: Consumer Strategies" report to their offering. ... Pain ... approach and treat their physical pain, emphasizing consumer survey analysis, ... pain sufferers and adults who have selected illnesses/conditions strongly associated ...
(Date:3/24/2017)... FinancialBuzz.com News Commentary   ... Medical cannabis products around the world are projected to gain popularity. A ... market will reach a value of USD 55.8 billion by 2025. ... growing industry. By the end of 2016, 28 states have now laws ... conservative states like Arkansas and North ...
Breaking Medicine Technology: