Navigation Links
New research demonstrates bone-marrow derived stem cells can reverse genetic kidney disease

The discovery that bone-marrow derived stem cells can regenerate damaged renal cells in an animal model of Alport syndrome provides a potential new strategy for managing this inherited kidney disease and offers the first example of how stem cells may be useful in repairing basement membrane matrix defects and restoring organ function.

Led by researchers at Beth Israel Deaconess Medical Center (BIDMC), the findings are described in the Proceedings of the National Academy of Sciences (PNAS), which appears on-line the week of April 24, 2006.

Symptoms of Alport syndrome, the second most common genetic cause of kidney failure, usually appear in children, affecting the kidneys' filtration system and typically leading to end-stage renal disease in the patient's teens, 20s or 30s. The disease additionally causes deafness in some patients.

"This is one of 31 human diseases that occur because of genetic defects in the body's extracellular matrix and basement membrane proteins, " explains the study's senior author Raghu Kalluri, PhD, chief of the division of matrix biology at BIDMC and associate professor of medicine at Harvard Medical School.

Present throughout the body, the extracellular matrix (ECM) is made of collagens, proteogylcans, noncollagenous glycoproteins and in some tissues, elastin fibers. The ECM serves a unique role by constructing a "scaffold" for cells, thereby maintaining the structural integrity of many tissues.

"In normal kidneys, a specialized form of extracellular matrix known as the glomerular basement membrane [GBM], composed primarily of type IV collagen, is the key component of the blood filtration apparatus," says Kalluri.

However, in patients with Alport syndrome, mutations in three different type IV collagen genes cause structural damage to the GBM, leading to a breakdown of the organ's filtration system, and resulting in the seepage of blood and protein into the urine and eventual kidney failure.< /p>

"With no known cure, treatment options for Alport syndrome are limited to kidney transplantation or lifelong dialysis," he adds. "Our lab set out to determine if bone marrow-derived stem cell therapy might provide another treatment option."

Using a mouse model of the disease, in which COL4A3 ?one of the three type IV collagen genes that is mutated in Alport patients -- had been removed, the researchers transplanted allogenic bone marrow into the knockout mouse.

According to Kalluri, within a period of about four weeks, the investigators found that approximately 10 percent of the transplanted cells had incorporated into the damaged regions of the kidney glomeruli in the knockout mouse, emerging as healthy renal cells (podocytes and mesangial cells). This led to clear improvement in the animal's kidney function and repair of the glomerular architecture defects.

"These results offer a possible therapeutic opportunity for both children and adults with Alport syndrome," he says. In addition, the new findings demonstrate that bone-marrow-derived stem cells can be used to repair extracellular/basement membrane defects, and according to Kalluri, the researchers' next step will be to see if the use of embryonic stem cells and circulating adult stem cells will achieve the same function.

"This new evidence offers hope that this is a treatment strategy that can be explored for other diseases that result from extracellular matrix defects," he notes.

Study coauthors include BIDMC researchers Hikaru Sugimoto, MD, PhD, Thomas Mundel, MD, PhD, Malin Sund, MD, PhD, and Liang Xie, PhD, and Dominic Cosgrove of Boystown National Research Hospital, Omaha, Nebraska.


'"/>

Source:Beth Israel Deaconess Medical Center


Related biology news :

1. Columbia research lifts major hurdle to gene therapy for cancer
2. U of M researcher examines newly emerging deadly disease
3. NYU researchers simulate molecular biological clock
4. First atlas of key brain genes could speed research on cancer, neurological diseases
5. New research questions basic tenet of neuron function
6. Vital step in cellular migration described by UCSD medical researchers
7. ASU researchers finds novel chemistry at work to provide parrots vibrant red colors
8. UCSD researchers maintain stem cells without contaminated animal feeder layers
9. Why do insects stop breathing? To avoid damage from too much oxygen, say researchers
10. New protein discovered by Hebrew University researchers
11. First real-time view of developing neurons reveals surprises, say Stanford researchers
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:5/3/2016)...  Neurotechnology, a provider of high-precision biometric identification ... Identification System (ABIS) , a complete system for ... can process multiple complex biometric transactions with high ... face or iris biometrics. It leverages the core ... MegaMatcher Accelerator , which have been used in ...
(Date:4/19/2016)... The new GEZE SecuLogic access ... "all-in-one" system solution for all door components. It can ... door interface with integration authorization management system, and thus ... minimal dimensions of the access control and the optimum ... offer considerable freedom of design with regard to the ...
(Date:3/31/2016)...   LegacyXChange, Inc. ... LegacyXChange is excited to release its first ... be launched online site for trading 100% guaranteed authentic ... also provide potential shareholders a sense of the value ... industry that is notorious for fraud. The video is ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... June 23, 2016 , ... In a new case report published today ... a patient who developed lymphedema after being treated for breast cancer benefitted from an ... paradigm for dealing with this debilitating, frequent side effect of cancer treatment. ...
(Date:6/23/2016)... , June 23, 2016  Blueprint Bio, a ... discoveries to the medical community, has closed its Series ... Matthew Nunez . "We have received a ... the capital we need to meet our current goals," ... provide us the runway to complete validation on the ...
(Date:6/23/2016)... On Wednesday, June 22, 2016, the NASDAQ Composite ... Jones Industrial Average edged 0.27% lower to finish at 17,780.83; ... has initiated coverage on the following equities: Infinity Pharmaceuticals Inc. ... NKTR ), Aralez Pharmaceuticals Inc. (NASDAQ: ARLZ ), ... more about these stocks by accessing their free trade alerts ...
(Date:6/23/2016)... Prairie, WI (PRWEB) , ... June 23, 2016 ... ... consultancy focused on quality, regulatory and technical consulting, provides a free webinar ... is presented on July 13, 2016 at 12pm CT at no charge. , ...
Breaking Biology Technology: