<unk> , also called lysosomal<unk> -mannosidase deficiency, is a disorder of oligosaccharide metabolism caused by decreased activity of the enzyme<unk> -mannosidase. This enzyme is coded for by the gene MANBA, located at 4q22-25.<unk>  is inherited in an autosomal recessive manner. Affected individuals appear normal at birth, and can have a variable clinical presentation. Infantile onset forms show severe neurodegeneration, while some children have intellectual disability. Hearing loss and angiokeratomas are common features of the disease.
Beta-mannosidosis
Alpha-mannosidosis
Galactosialidosis
Aspartylglucosaminuria
Glycosuria
Essential fructosuria
Alkaptonuria
Hawkinsinuria