據(jù)7月2日的《科學(xué)》雜志（Science）報道說,，一項在小鼠中的研究披露，在新生兒寶寶中的伴隨某種特別形式的糖尿病的肌無力,，其本原是神經(jīng)元性的,，這一發(fā)現(xiàn)可能會給患者帶來更為安全的療法,。 在新生兒中，iDEND是一種特別類型的糖尿病,，它常常伴隨有虛弱、松弛的肌肉以及發(fā)育上的延緩,。 這種情況是由KATP通道的突變所引起的,，KATP通道是細(xì)胞膜上的一種可調(diào)節(jié)鉀及激素分泌（諸如從胰腺分泌胰島素）的孔隙。
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通過研究僅在其肌肉或其神經(jīng)上表達(dá)突變的KATP基因的小鼠,，Rebecca Clark及其同僚發(fā)現(xiàn),，在iDEND中的運動損害源自于在中樞神經(jīng)系統(tǒng)（而非肌肉）中的該通道的錯誤激活。
罹患iDEND的病人常常接受磺脲的治療,，這是一種可阻斷腦中及肌肉中KATP通道的藥物,，而這些藥物可對心肌造成不良反應(yīng)。 這些新的發(fā)現(xiàn)提示,，對腦中KATP通道具有更大特異性的藥物可能會是治療的一種更為安全的選擇,。(生物谷Bioon.net)
生物谷推薦原文出處：
Science DOI: 10.1126/science.1186146
Muscle Dysfunction Caused by a KATP Channel Mutation in Neonatal Diabetes Is Neuronal in Origin
Rebecca H. Clark,1 James S. McTaggart,1,* Richard Webster,2,* Roope Mannikko,1,* Michaela Iberl,1 Xiuli Sim,1 Patrik Rorsman,3 Maike Glitsch,1 David Beeson,2 Frances M. Ashcroft1,
Gain-of-function mutations in Kir6.2 (KCNJ11), the pore-forming subunit of the KATP channel, cause neonatal diabetes. Many patients also suffer from hypotonia (weak and flaccid muscles) and balance problems. While the diabetes arises from suppressed insulin secretion by overactive KATP channels in pancreatic β cells, the source of the motor phenotype is unknown. Using mice carrying a human Kir6.2 mutation (V59M) targeted to either muscle or nerve, we show that analogous motor impairments originate in the central nervous system rather than in muscle or peripheral nerves. We also identify locomotor hyperactivity as a feature of KATP channel overactivity. These findings suggest that drugs targeted against neuronal, rather than muscle, KATP channels are needed to treat the motor deficits and that such drugs require high blood-brain barrier permeability.
1 Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford, OX1 3PT, UK.
2 Weatherall Institute of Molecular Medicine, University of Oxford, Oxford OX3 9DS, UK.
3 Oxford Centre for Diabetes Endocrinology and Metabolism, Churchill Hospital, Oxford OX3 7LJ, UK.
