carnitine function and requirement

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1、ABSTRACTL-Carnitinehasbeendescribedasa“con-mitochondriaforutilizationinenergy-generatingprocesses.ditionallyessential”nutrientforhumans.SegmentsoftheLong-chainfattyacids,ascoenzymeAesters,aretrans-humanpopulationsuggestedashavingarequirementforesterifiedtoL-carniti

2、neinareactioncatalyzedbycarnitinecarnitineincludeinfants(prematureandfull-term),pa-palmitoyltransferaseI(E.C.2.3.1.21)ofthemitochondrialtientsonlong-termparenteralnutrition,andperhapsoutermembrane.Long-chainacylcarnitineestersenterintochildren.Theevidencetosupportt

3、heseclaimsincludesmitochondriaviaaspecificcarrier,carnitine-acylcarnitine1)lowcirculatingcarnitineconcentrations;2)abnormaltranslocase.Onthematrixsideoftheinnermitochondrial(oratleastdifferent)circulatingmetaboliteconcentra-membranethelong-chainfattyacidistranseste

4、rifiedtotions(freefattyacids,triglycerides,ketonebodies),andintramitochondrialcoenzymeA,catalyzedbycarnitinepal-3)verylimitedandinconsistentgrowthdata.AnumbermitoyltransferaseII(E.C.2.3.1.21).Carnitinemayexittheofsubjectiveobservationsandanecdotalcasereportshavemit

5、ochondrionassuchorasashort-chainacylcarnitinebeenofferedinsupportofarequirementforcarnitine.ester,viathetranslocase.Thisfunctionofcarnitineisobliga-Exogenouscarnitineisrequiredtomaintain“normal”(intory:long-chainfattyacidscannotentermitochondriain-theepidemiologics

6、ense)plasmaorserumcarnitinecon-dependentoftranslocationasanesterofcarnitine.centrationsinhumansofallages.But“functionalcarni-L-Carnitinealsofacilitatesremovalfrommitochondriaoftinedeficiency:’definedbyabnormalclinicalpresentationshort-chainandmedium-chainfattyacids

7、thataccumulatecorrectablebycarnitineadministration,hasnotbeenasaresultofnormalandabnormalmetabolism.Short-anddemonstratedinanotherwisenormal(nonpathologic)medium-chainacids,asacyl-CoAestersarisingfrom(3-population.Ontheotherhand,nutritionalorpharmaco-oxidationandot

8、hermitochondrialprocesses,aretrans-logicalinterventionwithcarnitineoritsestersmaybeesterifiedtocarnitinebytheactionofcarnitineacetyltrans-benefic