Higher body temperature alters key protein in autoinflammatory disorder jclinicalinvest
"We discovered that there's a threshold of enzyme activity. At about 20% activity, there is no disease. Disease starts to appear if enzyme activity falls below this threshold, when the effect on proteins really kicks in," says Professor Rogers.
Mice with 20% enzyme activity had very mild disease, while animals with 10% activity had clear signs of disease and higher levels of abnormal proteins. Importantly, raising the body temperature decreased enzyme activity to almost undetectable levels, leading to very high amounts of the abnormal proteins.
"We can start to use this information predictively; for example, by measuring the level of abnormal proteins in samples of blood we may be able to foresee the severity of the symptoms" says Professor Rogers."Clinicians could use this knowledge to help diagnose and manage the disease."called NLRP3 plays a role in the inflammatory process of MKD.
The new findings are welcomed by Natalie Billiard, parent of a 13-year-old girl living with a rare autoinflammatory disorder she was diagnosed with as a baby."Fifty years ago my daughter's illness was termed 'not compatible with life.' We have come a long way thanks to people like Professor Rogers and the research his team is doing. It's giving our kids a chance at life," she says.
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