mannosidosis

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An inherited disorder caused by the deficiency of an enzyme necessary for the metabolism of mannose and characterized by mental retardation, kyphosis, and an enlarged tongue, with the accumulation of mannose in the body tissues.

An inborn error of metabolism, in which inactivity or an inherited deficiency of mannosidase results in lysosomal accumulation of mannose-rich substrates. Deficiency of the α-isomer (α-mannosidosis) is inherited in Aberdeen Angus, Murray Grey, Simmental, Holstein and Galloway calves as an autosomal recessive trait, and the resulting disease is of economic importance. From several months of age, affected calves show ataxia, head tremor, aggression, and finally paralysis. A similar disease has been reported in cats.
Deficiency of the β-isomer is inherited, also as an autosomal recessive trait, in Nubian goats and Saler calves, causing neurological deficits and tremors from birth. There are also skeletal abnormalities.

• acquired m. — caused by ingestion of astragalus, oxytropis and swainsona spp., which contain alkaloids that inhibit α-mannosidase activity. Called also locoweed poisoning.