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Identification of role for proteins in children's muscle disease could open up new treatment options
June 22, 2006A study presented by Mrs. Elisabeth Elst today shows for the first time that a protein—heat shock protein 60 (HSP60) — that is present in chronic inflammations, triggers a response by T-cells (a type of white blood cells that plays a part in the body's own immune response) in children with juvenile dermatomyositis (JDM).
The specific response has earlier been observed in juvenile idiopathic arthritis, but to date, little is known about the role of HSP60 in inflammatory myositis. Inflammatory myositis (IM) is the name given to a group of diseases that cause inflammation in the muscles of the body, which is mediated by the immune system of the body. The main symptoms are pain and weakness and can cause patient disability because of damage to the muscles. The main types of IM are dermatomyositis and polymyositis.
For children, the conditions of myositis are complex and are characterized by muscle damage due to an inflammatory process of the blood vessels that lie under the skin and muscles. Some of the symptoms include skin changes around the eyelids and over the knuckles and finger joints, as well as weakness in muscles, mainly affecting the large muscles around the hips and shoulders resulting in increased difficulty with walking, climbing stairs, getting up from the floor and lifting the arms. The children also often become uncharacteristically miserable and fractious and they may complain of tummy pain.
Heat shock proteins (HSP) are a group of proteins whose expression is increased when the cells are exposed to elevated temperatures. Production of high levels of heat shock proteins can also be triggered by exposure to different kinds of environmental stress conditions, such as infection, inflammation, exposure of the cell to toxins (e.g. ethanol, arsenic, and ultraviolet light), or water deprivation.
Mrs. Elst, pediatric immunologist at the University Medical Centre Utrecht, said, "We have shown for the first time that HSP60 plays an active part in the control of the inflammatory process in JDM. Thus, therapy aimed at the expansion of T-cells with regulatory capacities reacting to HSP60 could contribute to disease remission in patients with JDM. This conclusion opens up new perspectives for the understanding and approach for antigens in immunotherapy."
European League Against Rheumatism
Heat shock proteins (HSP) are a group of proteins whose expression is increased when the cells are exposed to elevated temperatures. Production of high levels of heat shock proteins can also be triggered by exposure to different kinds of environmental stress conditions, such as infection, inflammation, exposure of the cell to toxins (e.g. ethanol, arsenic, and ultraviolet light), or water deprivation.
Mrs. Elst, pediatric immunologist at the University Medical Centre Utrecht, said, "We have shown for the first time that HSP60 plays an active part in the control of the inflammatory process in JDM. Thus, therapy aimed at the expansion of T-cells with regulatory capacities reacting to HSP60 could contribute to disease remission in patients with JDM. This conclusion opens up new perspectives for the understanding and approach for antigens in immunotherapy."
European League Against Rheumatism
Muscle Disease Current Events and Muscle Disease News RSSSun exposure may trigger certain autoimmune diseases in women
Ultraviolet (UV) radiation from sunlight may be associated with the development of certain autoimmune diseases, particularly in women.
Australian team reveals world-first discovery in a 'floppy baby' syndrome
In a world first, West Australian scientists have cured mice of a devastating muscle disease that causes a Floppy Baby Syndrome - a breakthrough that could ultimately help thousands of families across the globe.
Nervous system may be culprit in deadly muscle disease
Brain may win out over brawn as the primary cause of breathing problems in children with a severe form of muscular dystrophy known as Pompe disease.
Researchers identify gene associated with muscular dystrophy-related vision problems
Skeletal muscle disease and vision deficits might seem unrelated, but a frog model of muscular dystrophy shows it is not such a leap.
Researchers develop DNA 'patch' for canine form of muscular dystrophy
Using a novel genetic technology that covers up genetic errors, researchers funded in part by the National Institutes of Health have developed a successful treatment for dogs with the canine version of Duchenne muscular dystrophy, a paralyzing, and ultimately fatal, muscle disease.
New potential therapeutic target discovered for genetic disorder -- Barth syndrome
Researchers at NYU Langone Medical Center may have discovered a new targeted intervention for Barth Syndrome (BTHS). BTHS, a sometimes fatal disease, is a serious genetic disorder occurring predominantly in males that leads to infection or heart failure in childhood.
'Smart scaffolds' may help heal broken hearts
Imagine new treatments for heart disease or muscle loss that direct the body to repair damaged tissue rather than helping it cope with a weakened condition.
Penn researchers discover 'modus operandi' of heart muscle protein
Researchers at the University of Pennsylvania School of Medicine have discovered that a protein called leiomodin (Lmod) promotes the assembly of an important heart muscle protein called actin. What's more, Lmod directs the assembly of actin to form the pumping unit of the heart. The findings appear in this week's issue of Science.
Lithium chloride slows onset of skeletal muscle disorder
A new UC Irvine study finds that lithium chloride, a drug used to treat bipolar disorder, can slow the development of inclusion body myositis, a skeletal muscle disease that affects the elderly.
Investigational drug tested for preventing muscle fiber death in muscular dystrophy
An investigational antiviral drug currently undergoing human trials in Europe for treating Hepatitis C infections may have potential to reduce muscle cell damage in Duchenne and other forms of muscular dystrophy (MD).
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