Maltase

Maltase pancreas, small intes tine, yeast maltose glucose 6-1 (gut) 6 6 (yeast)... 

Wort. Wort is the Hquid drained off the mash tun containing maltose, a grain sugar derived from the conversion of starch during the mashing process by the action of the organic enzyme, maltase, found in badey malt. 

Many enzymes are extremely specific. For example, the enzyme maltase catalyzes the hydrolysis of maltose ... 

This is the only function of maltase, but it is one that no other enzyme can perform. Many such digestive enzymes are required for the metabolism of carbohydrates, proteins, and fats. It has been estimated that without enzymes, it would take upward of 50 years to digest a meal. 

This reaction can be reversed either by using the enzyme maltase as a catalyst or by heating with acid. Two molecules of glucose are farmed when this happens. 

Eleven defects in the metabolism of glycogen have been reported nine of them affect skeletal muscle directly (see Figure 5), but only glycogenosis type II (acid maltase deficiency) and glycogenesis type V (myophosphorylase deficiency) are reasonably common the rest are rare and some have been recorded in isolated case studies only. 

Figure 6. Glycogen storage in acid maltase (AM) deficiency in this late-onset case not all muscle fibers are affected.

Among the eight possible diastereoisomers of carba-trehalose of type C, only that isomer having the same configuration as the natural trehalose was found to be a substrate of certain maltases or trehalases. ... 

Type II Pompe s disease Deficiency of lysosomal a-1 4- and 1 ->6-glucosldase (acid maltase) Fatal, accumulation of glycogen in lyso-somes, heart failure. 

Martiniuk, F., Chen, A., Donnabella, V. et al. (2000) Correction of glycogen storage disease type II by enzyme replacement with a recombinant human acid maltase produced by over-expression in a CHO-DHFR(neg) cell line. Biochemical and Biophysical Research Communications, 276 (3), 917-923. 

Human creatine kinase -MM MAK33 IgGl Cardiac disease, mitochondrial disorders, inflammatory myopathies, myasthenia, polymyositis, McArdle s disease, NMJ disorders, muscular dystrophy, ALS, hypo and hyperthyroid disorders, central core disease, acid maltase deficiency, myoglobinuria, rhabdomyolysis, motor neuron diseases, A. thaliana A. thaliana 2S2 seed storage protein SP + 0.02-0.4% TSP of fresh leaf extract (10-12% TSP of intercellular fluid) 52... 

An additional cause of weakness may be involvement of the anterior horn cells of the spinal cord, which is very conspicuous in infantile acid maltase deficiency. All three glycogenoses causing weakness are in fact due to generalized enzyme defects, but histological signs of denervation are not evident. 

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