A
tiny protein seems to be to blame for destroying brain cells in
patients suffering from the devastating, incurable Huntington's
disease, a study said Thursday.
罹患亨汀頓舞蹈症十分痛苦，而且目前尚未找到治療的方法，週四一項研究指出，某種微小的蛋白質似乎是破壞患者腦細胞的元兇。
Another faulty protein, called "huntingtin," has long been known to be the cause of Hungtington's Disease.
另一種名為「huntingtin」的異常蛋白質，向來被視為誘發亨汀頓舞蹈症的病因。
But
now researchers at Johns Hopkins University in Baltimore say they have
finally worked out why the huntingtin protein accumulates in cells
everywhere, but only kills cells in the part of the brain that controls
movement.
不過現在，巴爾的摩約翰霍普金斯大學的研究人員表示，他們終於找出原因，可以解釋為什麼huntingtin蛋白質會囤積在全身的細胞中，但卻只會殺死腦中某部分控制動作的細胞。
They
said in the study published Thursday in Science magazine that the tiny
protein, Rhes, only found in the brain cells which cause movement
seemed to be to blame.
週四發表於《科學雜誌》的研究中，研究人員指出，「Rhes」這種只在腦部掌管活動的細胞中找得到的微小蛋白質，似乎是致病的元兇。
"It's
always been a mystery why, if the protein made by the HD gene is seen
in all cells of the body, only the brain, and only a particular part of
the brain, the corpus striatum, deteriorates," said Solomon Snyder,
professor of neuroscience at Johns Hopkins.
約翰霍普金斯大學的神經科學教授索羅門史奈德表示：「如果HD（亨汀頓舞蹈症）基因所製造的蛋白質，在全身的細胞裡都有，那為什麼只有腦部，而且是腦部某個特定的部位（紋狀體）會退化？這問題一直都是一個謎。」
"By
finding the basic culprit, the potential is there to develop drugs that
target it and either prevent symptoms or slow them down."
「由於找到了根本的病因，就有可能研發專門的藥物，以預防或減緩這種疾病的症狀。」
Carrying
out tests on sufferers and on the brain cells of mice induced with the
disease, the team found that the Rhes protein attached itself more
strongly to the mutant huntingtin protein than proteins in other cells.
研究小組針對患者施行檢測，並檢驗受HD誘發的老鼠腦細胞，發現Rhes蛋白質會更強力附著在突變的huntingtin蛋白質上，附著在其他細胞中蛋白質上的狀況則較輕微。
And within 48 hours the affected brain cells had died.
染病的腦細胞在48小時之內就死亡了。
Huntington's
is caused by a genetic defect, passed down from parent to child, which
produces a mutant version of the huntingtin protein that gathers in all
cells of the body.
亨汀頓舞蹈症是由基因缺陷所引起，父母親會遺傳給子女，這種基因缺陷會製造突變種的huntingtin蛋白質，堆積在全身的細胞之中。
But
it is its effects on the brain that causes patients to suffer
irreversible loss of the ability to their control movements, eventual
mental impairment, behavioral problems and death.
但是huntingtin蛋白質會對腦部產生影響，導致患者掌管活動的能力出現無法補救的損失，最終造成患者心智受損、行為問題甚至死亡。
"Now
that we've uncovered the role of Rhes, it's possible that drugs can be
designed that specifically target Rhes to treat or even prevent the
disease," said Snyder.
史奈德說：「現在我們既然已經發現了Rhes產生的影響，就有可能研發專門對付Rhes的藥物，用以治療或甚至預防亨汀頓舞蹈症。」
