Mannosidosis
Encyclopedia
Mannosidosis is a deficiency in mannosidase
, an enzyme
.
There are two types:
Mannosidase
Mannosidase is an enzyme which hydrolyzes mannose.There are two types:* alpha-Mannosidase* beta-MannosidaseA deficiency is associated with mannosidosis....
, an enzyme
Enzyme
Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...
.
There are two types:
- Alpha-mannosidosisAlpha-mannosidosisAlpha-mannosidosis is a lysosomal storage disorder caused by deficient activity of the enzyme alpha-D-mannosidase . In humans it is known to be caused by an autosomal recessive genetic mutation...
- Beta-mannosidosisBeta-mannosidosisBeta-mannosidosis, also called lysosomal beta-mannosidase deficiency, is a rare autosomal recessiveform of mannosidosis associated with MANBA....