Navigation Links
Investigational drug tested for preventing muscle fiber death in muscular dystrophy
Date:3/16/2008

o be flooded by calcium and reorganize, swell and eventually rupture. This triggers cell death in muscle fibers and leads to the progressive muscle weakness, wasting and often early death associated with muscular dystrophy.

Mice lacking the protein delta-sarcoglycan exhibited severe dystrophy and swelling in both skeletal and heart muscle. When Dr. Molkentin and his colleagues deleted the gene encoding cyclophilin D in these mice, the muscle cells returned to near normal and did not show appreciable signs of swelling and cell death. The investigators repeated the experiment with mice lacking a gene encoding laminin alpha2, which causes a more severe dystrophy, swollen skeletal muscle cells and premature death before the mice reach two months of age. In contrast, mice lacking both laminin alpha2 and cyclophilin D showed much healthier muscle cells, increased body weight and walked more. Also, 75 percent of the mice lacking laminin alpha2 and cyclophilin D lived more than three times longer than mice lacking only laminin alpha2.

These findings led the research team to look for pharmacological treatments that also could inhibit cyclophilin D. The drug cyclosporine is a well-documented inhibitor of the protein, but its use is problematic because it also inhibits a protein, calcineurin, crucial to skeletal muscle cell repair after injury and to the development of skeletal muscle cells. The advantage of Debio-025 is that while it inhibits cyclophilin D and blocks cell death in a number of situations, the drug does not suppress the immune system or block calcineurin. The drug is manufactured by DebioPharm S.A. of Lausanne, Switzerland, which provided Debio-025 for use in the study.

The researchers also found their study may have implications beyond skeletal muscle disease as cyclophilin D deletion reduced cardiac dysfunction caused by calcium-overload induced necrosis. This led the team to suggest that mitochondrial-dependent necrosis may also
'/>"/>

Contact: Nick Miller
513-803-6035
Cincinnati Children's Hospital Medical Center
Source:Eurekalert

Page: 1 2 3

Related medicine news :

1. Enrollment completed in 2 pivotal phase III studies of the investigational cancer drug vandetanib
2. New Drug Application Submitted to FDA for Investigational Analgesic Tapentadol Immediate Release Tablets
3. UCB Announces Positive Results From Phase II Clinical Trial of Investigational Anti-Epilepsy Drug Brivaracetam
4. SGX Pharmaceuticals Submits Investigational New Drug Application for SGX523, a Highly Potent, Selective, Orally Bioavailable cMET Inhibitor
5. MetaCure(TM) Launches Investigational Study of Gastric Stimulator for Patients With Type 2 Diabetes Who are Overweight
6. Abbotts Investigational SIMCOR(R) Offers Comparable LDL Lowering to Simvastatin and Significantly Raises HDL and Lowers Triglycerides in Phase III Study
7. Dynavax and Merck & Co., Inc. Announce Partnership to Develop HEPLISAV(TM), an Investigational Hepatitis B Vaccine Currently in Phase 3
8. Ono and Medarex Announce Allowance of Investigational New Drug Application for Fully Human Anti-PD1 Antibody
9. Supratek Pharma Inc. announced today that the U.S. Food and Drug Administration (FDA) has cleared its Investigational New Drug (IND) application for SP1049C for the treatment of metastatic adenocarcinoma of the upper gastrointestinal tract
10. Accentia announces investigational new drug application for Revimmune for refractory MS
11. FDA Accepts New Drug Application for Priority Review of Investigational HIV Treatment TMC125
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/30/2015)... Las Vegas, NV (PRWEB) , ... June 30, ... ... has recently returned to the U.S. after a 5-day tour in India. ... that are currently collaborating with Trivedi Master Wellness™ and Transformational Leader/Internationally ...
(Date:6/30/2015)... ... June 30, 2015 , ... ... service and repair companies in San Diego. Bill Howe offers all employees ample ... through several organizations, but most notably through the local Plumbing-Heating-Cooling-Contractors of San Diego ...
(Date:6/30/2015)... (PRWEB) , ... June 30, 2015 , ... Some may ... – and the ongoing efforts of New York State Athletic Trainers’ Association (NYSATA) and ... NYSATA was recognized with two national awards, and a number of members also received ...
(Date:6/30/2015)... ... June 30, 2015 , ... ... held October 28-31, 2015 at Disney’s Coronado Springs Resort in Lake Buena Vista, ... networking. Each year, this premiere event for psychiatric-mental health nursing draws more than ...
(Date:6/30/2015)... ... 2015 , ... Medflow, the leader in the ophthalmic EHR ... system from the company exclusively dedicated to helping eye care professionals for over ... solution combining modern cloud technology with Medflow’s proven expertise in the eye care ...
Breaking Medicine News(10 mins):Health News:Trivedi Global Inc. CEO Visits India to Finalize Science Projects to Create Anti-Aging, Overall Health and Probiotic Products with Help of a Multi-Billion Dollar Company 2Health News:Trivedi Global Inc. CEO Visits India to Finalize Science Projects to Create Anti-Aging, Overall Health and Probiotic Products with Help of a Multi-Billion Dollar Company 3Health News:Two Local San Diego Plumbers from Bill Howe Plumbing, Inc. Graduate from PHCC Academy 2Health News:Two Local San Diego Plumbers from Bill Howe Plumbing, Inc. Graduate from PHCC Academy 3Health News:NYSATA and Members Recognized with National Awards 2Health News:NYSATA and Members Recognized with National Awards 3Health News:American Psychiatric Nurses Association 29th Annual Conference: Continuing Education and Collaboration in Psychiatric-Mental Health and Nursing 2Health News:American Psychiatric Nurses Association 29th Annual Conference: Continuing Education and Collaboration in Psychiatric-Mental Health and Nursing 3Health News:Medflow Launches MEDFLOW 2.0 EHR – The Cloud-Based Ophthalmology EHR 2
... The North Desert Village landscaping experiment was conducted in ... of this experiment were that the graduate student families ... and fertile lawns and ornamental bushes spend time together ... in a nearby cluster of six homes on native ...
... New Orleans – The manner with which an individual ... rather than the chronological age, say leading researchers in ... is all about handling stress well and having a ... of studies on how stress hormones affect the brain, ...
... H.Influenza could be virtually abolished in Africa by routinely ... from the Oxford University have shown in their research ... H. influenza Type b (Hib) significantly reduced invasive Hib ... their findings in the Journal of the American Medical ...
... the UN Food and Agriculture Organisation (FAO) and the World ... an early warning system in order to spread awareness of ... animals to humans. ,Such a system has become imperative ... with alarming frequency. "The spread of avian flu reinforces the ...
... can also cause problems in men, according to a new ... at the Telethon Institute for Child Health Research // said ... in baby boys was now important. ,"The common ... affects girls, and that the genetic flaw would be so ...
... Institute in Sweden haves shown that the nerve cells of the ... ,The researchers explained that in a human body most of the ... example the cells of the liver take an approximate of two ... however found in their studies that the cells of the brain ...
Cached Medicine News:Health News:Environmental Surrounding Affects Human Behaviour 2Health News:Studying the psycho neurobiology of aging 2Health News:Studying the psycho neurobiology of aging 3Health News:Researchers claim That Regular Use Of Hib Vaccine In Africa Could Eliminate H. Influenza totally. 2Health News:Researchers claim That Regular Use Of Hib Vaccine In Africa Could Eliminate H. Influenza totally. 3Health News:Rett Syndrome In Men? 2Health News:Studies Show That Brain Cells Do Not Regenerate But Remain The Same Throughout 2
(Date:6/29/2015)... VILLAGE, Nev. , June 29, 2015  PDL ... it has entered into a credit agreement with CareView ... with up to $40 million of secured debt financing. ... industry focused on patient care monitoring. Under ... to $40 million of debt financing in two tranches ...
(Date:6/29/2015)... NESS ZIONA, Israel , ... Ltd. (Nasdaq: BVXV, TASE: BVXV) today announced positive preliminary ... M-001, BiondVax,s candidate for a universal influenza vaccine. In ... the ages of 50 to 65, followed by an ... 3 weeks later. M-001 was found to be safe ...
(Date:6/29/2015)... , June 29, 2015 DURECT ... James E. Brown , President and CEO, ... Conference on Wednesday, July 8 at 11:45 a.m. ... Le Parker Meridien Hotel in New York City.  ... may request a one-on-one meeting through the conference ...
Breaking Medicine Technology:PDL BioPharma Announces $40 Million Financing Transaction with CareView Communications 2PDL BioPharma Announces $40 Million Financing Transaction with CareView Communications 3PDL BioPharma Announces $40 Million Financing Transaction with CareView Communications 4BiondVax Presents BVX-006 Preliminary Phase II Results: the Universal Flu Vaccine at Higher Doses is Safe and Improves Immunogenicity in Older Adults 2BiondVax Presents BVX-006 Preliminary Phase II Results: the Universal Flu Vaccine at Higher Doses is Safe and Improves Immunogenicity in Older Adults 3DURECT to Present at the Cantor Fitzgerald Healthcare Conference 2
... risk of,stroke by 34% in patient with atrial fibrillation or ... adequately treated by antithrombotic ... a,post-hoc analysis of the data from the ATHENA study were ... Society of Cardiology,congress 2008, in Munich, Germany. Previous results from ...
... in Subjects with ALS, RICHMOND, Calif., Sept. 3 ... that it has opened a Phase,2 clinical trial (SB-509-801) ... progressive, degenerative motor-neuron disease for which there,are limited treatment ... an injectable formulation of a plasmid,encoding a zinc finger ...
Cached Medicine Technology:Further Analysis From ATHENA Study Showed That Multaq(R) (dronedarone) Reduced the Risk of Stroke in Patients With Atrial Fibrillation 2Further Analysis From ATHENA Study Showed That Multaq(R) (dronedarone) Reduced the Risk of Stroke in Patients With Atrial Fibrillation 3Further Analysis From ATHENA Study Showed That Multaq(R) (dronedarone) Reduced the Risk of Stroke in Patients With Atrial Fibrillation 4Further Analysis From ATHENA Study Showed That Multaq(R) (dronedarone) Reduced the Risk of Stroke in Patients With Atrial Fibrillation 5Sangamo BioSciences Initiates Phase 2 Clinical Trial of Treatment for Amyotrophic Lateral Sclerosis (ALS) 2Sangamo BioSciences Initiates Phase 2 Clinical Trial of Treatment for Amyotrophic Lateral Sclerosis (ALS) 3Sangamo BioSciences Initiates Phase 2 Clinical Trial of Treatment for Amyotrophic Lateral Sclerosis (ALS) 4Sangamo BioSciences Initiates Phase 2 Clinical Trial of Treatment for Amyotrophic Lateral Sclerosis (ALS) 5
... acetabular cup system has been designed to ... tri-spiked and cluster hole configurations. The objective ... micromotion on three primary modes: Tilt, migration ... features result in initial and long-term fixation. ...
Optimal solution for normal acetabulum....
Optimal solution for normal acetabulum....
Biopro rimmed acetabular is manufactured from titanium and is porous coated on the outside of its shell. The rimmed acetabular accommodates a polyethylene inert....
Medicine Products: