Urinary excretion of acid-soluble peptides in children with Duchenne muscular dystrophy

Urinary excretion of acid-soluble peptides in children with Duchenne muscular dystrophy

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1、ActaPaediatricaJaponica(1994)36,627-631OriginalArticleUrinaryexcretionofacid-solublepeptidesinchildrenwithDuchennemusculardystrophyKUMIKOHIRANOANDYOSHIMASASAKAMOTO'FacultyofScienceofLiving,OsakaCityUniversity,Osaka,JapanAbstractInordertoinvestigatethevalidityo

2、fthehypothesisthatacid-solublepeptides(ASP)inurinaryexcretacanbeappliedasanindexoftheproteincatabolismofthewholebody,wemeasuredtheurinaryexcretionofASPin46normalchildrenandin18childrenwithDuchennemusculardystrophy(DMD),inwhichcontinuousbreakdownofskeletalmuscl

3、eproteinispresumed.ThemeanvalueofASPinthechildrenwithDMDwassignificantlyhigherthanthatinnormalcontrols.TheconcentrationofASPwascorrelatedwiththatof3-methylhistidine(3MH),whichhasbeenproposedasanindexofmusclebreakdown.ThisfindingindicatesthaturinaryASPreflectst

4、hecatabolismofbodyproteins.NocorrelationwasobservedbetweentheconcentrationofASPandthatof1-methylhistidine(IMH),whichisusedasanobjectiveindexofmeatandfishingestion.Aftertheadministrationofbestatin,aninhibitorofleucineaminopeptidase,for9months,theurinaryASPconce

5、ntrationofchildrenwithDMDincreasedmarkedly.Thisincreaseisthoughttohavebeendirectlycausedbythebestatinitself.UrinaryASPisthereforeapparentlyamoreconvenientlyappliedindexofproteincatabolismthanisurinary3MH,whichrequirestheapplicationofseveralrestrictions.However

6、,itshouldnotbeappliedwhentheeffectofbestatinadministrationisevident.Keywordsbestatin,1-methylhistidine,3-methylhistidine,musculardystrophy,urinaryacid-solublepeptide.AlthoughclinicalassessmentofproteinmetabolismstatusIncontrast,urinary3-methylhistidine(3MH)exc

7、retionshouldbeconductedinpatientswithseveralclinicalisfrequentlyusedasanindicatorofmuscleproteinconditionsassociatedwithnitrogenloss,includingtrauma,breakdown.However,dietaryintakeofmeat,anexogenousinfection,malnutrition,starvation,andhormonalcon-sourceof3MH,i

8、sknowntoresultinincreasedurinaryditions,accurateassessmentisoftendifficult.excretionof3MH.3Recently,Noguchireportedthattheurinaryexcretion1-Methylhistidine(IMH)ispresentinmeatasaof

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