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1,4-α-glucan branching enzyme [EC 2.4.1.18] an enzyme of the transferase class that catalyzes the cleavage of internal α-1,4-glucoside linkages in glycogen (or, in plants, amylopectin) and transfer of the fragments into α-1,6 linkages, thus creating branches in the glycogen molecule. Deficiency of the enzyme, an autosomal recessive trait, results in glycogen storage disease, type IV. Called also brancher or branching enzyme.
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1,4-diethylene dioxide dioxane.
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1-phosphatidylinositol phosphodiesterase [EC 3.1.4.10] a phospholipase C specific for phosphoinositides, occurring in all tissues. It is part of a mechanism for mobilization of calcium in response to hormones; it is activated by hormonal binding via a G protein and begins the cycle of phosphoinositide metabolism. It can also play a role in the liberation of arachidonic acid from phosphatidylinositol.
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1-pyrroline-5-carboxylate dehydrogenase [EC 1.5.1.12] an enzyme of the oxidoreductase class that catalyzes the dehydrogenation of Δ1-pyrroline 5-carboxylate to glutamate, using NAD+ as an electron acceptor; the reaction is a step in the degradation of proline and excess ornithine. The enzyme can also oxidize the 3-hydroxy derivative of Δ1-pyrroline 5-carboxylate to form 4-hydroxyglutamate. Deficiency of the enzyme, an autosomal recessive trait, is the cause of hyperprolinemia, type II.
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11β-h. steroid 11β-monooxygenase.
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