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Bile acylcarnitine analysis revealed a similar acylcarnitine pattern as in serum (Figure 4B). However, the acylcarnitine profiles of the MCAD−/− mice are different from those of human MCAD-deficient patients (Figure 4C). Human patients present with elevated levels of C6, C8, and C10:1 acylcarnitines, as did the mutant mice; however, the predominant peak was C8 acylcarnitine in humans, whereas in the mouse it was C10:1 acylcarnitine."}

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{"project":"craft-ca-core-ex-dev","denotations":[{"id":"T3291","span":{"begin":694,"end":707},"obj":"CHEBI:17387"},{"id":"T3290","span":{"begin":675,"end":680},"obj":"NCBITaxon:10088"},{"id":"T3262","span":{"begin":135,"end":139},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3261","span":{"begin":129,"end":130},"obj":"SO_EXT:normal_or_wild_type_or_present"},{"id":"T3260","span":{"begin":127,"end":128},"obj":"SO_EXT:normal_or_wild_type_or_present"},{"id":"T3259","span":{"begin":123,"end":127},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3258","span":{"begin":90,"end":99},"obj":"CHEBI:17126"},{"id":"T3257","span":{"begin":84,"end":89},"obj":"UBERON:0001977"},{"id":"T3256","span":{"begin":34,"end":38},"obj":"NCBITaxon:10088"},{"id":"T3255","span":{"begin":32,"end":33},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3254","span":{"begin":30,"end":31},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3253","span":{"begin":26,"end":30},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3252","span":{"begin":20,"end":21},"obj":"SO_EXT:normal_or_wild_type_or_present"},{"id":"T3251","span":{"begin":18,"end":19},"obj":"SO_EXT:normal_or_wild_type_or_present"},{"id":"T3250","span":{"begin":14,"end":18},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3289","span":{"begin":652,"end":658},"obj":"NCBITaxon:9606"},{"id":"T3288","span":{"begin":635,"end":648},"obj":"CHEBI:17387"},{"id":"T3287","span":{"begin":592,"end":596},"obj":"NCBITaxon:10088"},{"id":"T3286","span":{"begin":585,"end":591},"obj":"SO_EXT:sequence_altered_entity_or_alteration_process"},{"id":"T3285","span":{"begin":558,"end":572},"obj":"CHEBI:17387"},{"id":"T3284","span":{"begin":493,"end":498},"obj":"NCBITaxon:9606"},{"id":"T3283","span":{"begin":456,"end":460},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3282","span":{"begin":450,"end":455},"obj":"NCBITaxon:9606"},{"id":"T3281","span":{"begin":417,"end":421},"obj":"NCBITaxon:10088"},{"id":"T3280","span":{"begin":415,"end":416},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3268","span":{"begin":159,"end":160},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3267","span":{"begin":157,"end":158},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3266","span":{"begin":153,"end":157},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3265","span":{"begin":143,"end":147},"obj":"NCBITaxon:10088"},{"id":"T3264","span":{"begin":141,"end":142},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3263","span":{"begin":139,"end":140},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3279","span":{"begin":413,"end":414},"obj":"SO_EXT:sequence_nullness_or_absence"},{"id":"T3278","span":{"begin":409,"end":413},"obj":"GO_PR_EXT:medium_chain_acyl_CoA_dehydrogenase"},{"id":"T3277","span":{"begin":379,"end":392},"obj":"CHEBI:17387"},{"id":"T3276","span":{"begin":347,"end":352},"obj":"UBERON:0001977"},{"id":"T3275","span":{"begin":319,"end":332},"obj":"CHEBI:17387"},{"id":"T3274","span":{"begin":277,"end":290},"obj":"CHEBI:17387"},{"id":"T3273","span":{"begin":272,"end":276},"obj":"UBERON:0001970"},{"id":"T3272","span":{"begin":237,"end":250},"obj":"CHEBI:17387"},{"id":"T3271","span":{"begin":204,"end":221},"obj":"CHEBI:86063"},{"id":"T3270","span":{"begin":198,"end":203},"obj":"UBERON:0001977"},{"id":"T3269","span":{"begin":161,"end":165},"obj":"NCBITaxon:10088"}],"text":"Comparison of MCAD+/+ and MCAD−/− mice revealed no significant differences in total serum carnitine concentrations between MCAD+/+ and MCAD−/− mice, but MCAD−/− mice had a 5- to 6-fold elevation of serum decenoylcarnitine evident in the acylcarnitine profile (Figure 4A). Bile acylcarnitine analysis revealed a similar acylcarnitine pattern as in serum (Figure 4B). However, the acylcarnitine profiles of the MCAD−/− mice are different from those of human MCAD-deficient patients (Figure 4C). Human patients present with elevated levels of C6, C8, and C10:1 acylcarnitines, as did the mutant mice; however, the predominant peak was C8 acylcarnitine in humans, whereas in the mouse it was C10:1 acylcarnitine."}