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논문 기본 정보

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Kristy Swiderski (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of) Rebecka Bindon (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of) Jennifer Trieu (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of) Timur Naim (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of) Shana Schokman (Enteric Nervous System Laboratory Department of Physiology The University of Melbourne Australia) Mathusi Swaminathan (Enteric Nervous System Laboratory Department of Physiology The University of Melbourne Australia) Anita J L Leembruggen (Enteric Nervous System Laboratory Department of Physiology The University of Melbourne Australia) Elisa L Hill-Yardin (Gut-Brain Axis Laboratory School of Health and Biomedical Sciences RMIT University Melbourne Austra) René Koopman (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of) Joel C Bornstein (Enteric Nervous System Laboratory Department of Physiology The University of Melbourne Australia) Gordon S Lynch (Centre for Muscle Research Department of Physiology School of Biomedical Sciences The University of)
저널정보
대한소화관운동학회(현 대한소화기능성질환.운동학회) Journal of Neurogastroenterology and Motility (JNM) Journal of Neurogastroenterology and Motility (JNM) Vol.26 No.1
발행연도
2020.1
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133 - 146 (14page)

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Background/Aims: Patients with Duchenne muscular dystrophy exhibit significant, ongoing impairments in gastrointestinal (GI) function likely resulting from dysregulated nitric oxide production. Compounds increasing neuronal nitric oxide synthase expression and/or activity could improve GI dysfunction and enhance quality of life for dystrophic patients. We used video imaging and spatiotemporal mapping to identify GI dysfunction in mdx dystrophic mice and determine whether dietary intervention to enhance nitric oxide could alleviate aberrant colonic activity in muscular dystrophy. Methods: Four-week-old male C57BL/10 and mdx mice received a specialized diet either with no supplementation (control) or supplemented (1 g/kg/day) with L-alanine, L-arginine, or L-citrulline for 8 weeks. At the conclusion of treatment, mice were sacrificed by cervical dislocation and colon motility examined by spatiotemporal (ST) mapping ex vivo. Results: ST mapping identified increased contraction number in the mid and distal colon of mdx mice on control and L-alanine supplemented diets relative to C57BL/10 mice (P < 0.05). Administration of either L-arginine or L-citrulline attenuated contraction number in distal colons of mdx mice relative to C57BL/10 mice. Conclusions: GI dysfunction in Duchenne muscular dystrophy has been sadly neglected as an issue affecting quality of life. ST mapping identified regional GI dysfunction in the mdx dystrophic mouse. Dietary interventions to increase nitric oxide signaling in the GI tract reduced the number of colonic contractions and alleviated colonic constriction at rest. These findings in mdx mice reveal that L-arginine can improve colonic motility and has potential therapeutic relevance for alleviating GI discomfort, improving clinical care, and enhancing quality of life in Duchenne muscular dystrophy.
#Arginine #Duchenne #Gastrointestinal diseases #Muscular dystrophy #Duchenne #Nitric oxide synthase type I #colon

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참고문헌 (59)

참고문헌 신청
Barohn RJ / 1988 / Gastric hypomotility in Duchenne’s muscular dystrophy / N Engl J Med 319:15~18 google schola Bensen ES / 1996 / Acute gastric dilatation in Duchenne muscular dystrophy : a case report and review of the literature / Arch Phys Med Rehabil 77:512~514 google schola Borrelli O / 2005 / Evolution of gastric electrical features and gastric emptying in children with Duchenne and Becker muscular dystrophy / Am J Gastroenterol 100:695~702 google schola Dinan D / 2003 / Gastric wall weakening resulting in separate perforations in a patient with Duchenne’s muscular dystrophy / AJR Am J Roentgenol 181:807~808 google schola Okan M / 1997 / Gastric emptying time in children with progressive muscular dystrophy / Turk J Pediatr 39:69~74 google schola

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