Enzymes branching enzyme

The glucose molecules are added on to the existing chain in such a way that they are joined in the 1 4a configuration and repetition of the process results in the production of the linear polymer amylose (Fig. 5.11). The synthesis of branched molecules of amylopection is achieved by the operation of another enzyme branching enzyme) which joins linear polymers in the 1 6a configuration (see p. 108 for information on the phosphorylase reaction which may also be involved in a minor way in the synthesis and degradation of 1 4a-polyglucans). 

Glycogenosis type IV (branching enzyme deficiency) results in the formation of a variant of glycogen, characterized by abnormally long inner and outer glucosyl chains and fewer branch points than normal. The abnormal variant is stored in sufficient amounts to cause some vacuolation. The clinical manifestations of this... 

Type IV Amylopectinosis, Andersen s disease Absence of branching enzyme Accumulation of a polysaccharide having few branch points. Death due to cardiac or liver failure in first year of life. 

Potassium trisiamylborohydride, 20 605 Potassium winchite, 3 289 Potassium-zirconium carbonate, 26 637 Potato branching enzyme, 12 493 Potatoes, citric acid in, 6 632t Potato fungicide, 13 57 Potato starch, 4 724t Potential energy diagrams, 10 118-119 Potential gum test, 12 400 Potentiated toxic effects, 25 214 Potentiometric measurements, 14 612 Potentiometric titrations, 9 585-58 ... 

Transaminase enzymes (also called aminotransferases) specifically use 2-oxoglutarate as the amino group acceptor to generate glutamate but some have a wide specificity with respect to the amino donor. For example, the three branched-chain amino acids leucine, isoleucine and valine, all serve as substrates for the same enzyme, branched-chain amino acid transaminase, BCAAT ... 

Branching enzyme is responsible for introducing a-1,6 linked branches into the granule as it grows. The process by which the branch is introduced is shown schematically in Figure 1-14-3. Branching enzyme ... 

IV Andersen (amylopectinosis) Branching enzyme Infantile hypotonia, cirrhosis, death by 2 years Very few branches, especially toward periphery... 

The proglycogen is extended to produce macroglycogen by action of glycogen synthase and the branching enzyme (Figure 6.18). 

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