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{"project":"craft-ca-core-dev","denotations":[{"id":"T3120","span":{"begin":416,"end":418},"obj":"PR:000006506"},{"id":"T3123","span":{"begin":484,"end":494},"obj":"PR:000004687"},{"id":"T3124","span":{"begin":511,"end":513},"obj":"PR:000006300"},{"id":"T3125","span":{"begin":556,"end":558},"obj":"PR:000006506"},{"id":"T3126","span":{"begin":575,"end":583},"obj":"CHEBI:15361"},{"id":"T3127","span":{"begin":575,"end":583},"obj":"_FRAGMENT"},{"id":"T3128","span":{"begin":604,"end":627},"obj":"GO:0045254"},{"id":"T3129","span":{"begin":588,"end":627},"obj":"GO:0045240"},{"id":"T3114","span":{"begin":4,"end":9},"obj":"UBERON:0000178"},{"id":"T3115","span":{"begin":10,"end":14},"obj":"CHEBI:22918"},{"id":"T3116","span":{"begin":241,"end":254},"obj":"GO:0017086"},{"id":"T3117","span":{"begin":348,"end":361},"obj":"CHEBI:50488"},{"id":"T3118","span":{"begin":376,"end":378},"obj":"PR:000006300"},{"id":"T3119","span":{"begin":387,"end":400},"obj":"CHEBI:50488"},{"id":"T3121","span":{"begin":474,"end":475},"obj":"_FRAGMENT"},{"id":"T3122","span":{"begin":486,"end":494},"obj":"PR:000004686"}],"relations":[{"id":"R339","pred":"_lexicallyChainedTo","subj":"T3122","obj":"T3121"},{"id":"R340","pred":"_lexicallyChainedTo","subj":"T3128","obj":"T3127"}],"text":"the blood BCAA levels are normal [1]. The overall incidence of MSUD in the general population is 1:185,000 [1], and in certain population groups, such as Mennonites of Pennsylvania, the incidence is estimated to be as high as 1:176 [2].\nThe BCKDH complex, the deficient enzyme in MSUD, consists of three catalytic proteins, a decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoyl dehydrogenase (E3). The E1 component is a heterotetramer composed of two α and two β subunits [3]. The E1 and E2 components are specific to BCKDH, whereas E3 is also used by pyruvate and α-ketoglutarate dehydrogenase complexes [3] and the glycine cleavag"}