Mannosidosis

From Cow
Typical ataxic gait in a cow with mannosidosis

Mannosidosis is a rare genetic lysosomal storage disease of cattle worldwide[1].

Lysosomal alpha-mannosidase is a broad specificity exoglycosidase involved in the ordered degradation of glycoproteins. Deficiencies in this enzyme result in global metabolic disorders in cattle[2].

There are two variants of this disease:

Cattle affected with α-mannosidosis show signs of abortion, neonatal deaths, and in young calves, ataxia, head tremor, aggression and failure to thrive[5]. Most affected calves die within the first year.

In Salers cattle affected by β-Mannosidosis, calves show signs of recumbency and ataxia.

Diagnosis of these diseases requires PCR assay sampling of suspect cattle[6].

There is no specific treatment for affected cattle, and crossbreeding in commercial beef production has helped reduce the significance of this recessive defects[7].

References

  1. Leipprandt JR et al (1999) Identification of a bovine beta-mannosidosis mutation and detection of two beta-mannosidase pseudogenes. Mamm Genome 10(12):1137-1141
  2. Heikinheimo P et al (2003) The structure of bovine lysosomal alpha-mannosidase suggests a novel mechanism for low-pH activation. J Mol Biol 327(3):631-644
  3. Berg T et al (1997) Molecular heterogeneity for bovine alpha-mannosidosis: PCR based assays for detection of breed-specific mutations. Res Vet Sci 63(3):279-282
  4. Taylor JF et al (1993) Estimation of genotype distributions and posterior genotype probabilities for beta-mannosidosis in Salers cattle. Genetics 135(3):855-868
  5. Faid V et al (2006) Site-specific glycosylation analysis of the bovine lysosomal alpha-mannosidase. Glycobiology 16(5):440-461
  6. Healy PJ & Malmo J (1998) Roles for biochemical and polymerase chain reaction technologies in diagnosis and control of bovine alpha-mannosidosis. Aust Vet J 76(10):699-700
  7. Healy PJ (1996) Testing for undesirable traits in cattle: an Australian perspective. J Anim Sci 74(4):917-922