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. 1974 Feb;137(2):363–371. doi: 10.1042/bj1370363

Mannosidosis in Angus cattle. The enzymic defect

N C Phillips 1, D Robinson 1, B G Winchester 1, R D Jolly 2
PMCID: PMC1166125  PMID: 4207387

Abstract

Normal calf α-mannosidase activity exists in at least three forms separable by chromatography on DEAE-cellulose and by starch-gel electrophoresis. Two components, A and B, have optimum activity between pH3.75 and 4.75, but component C has an optimum of pH6.6. Components A and B are virtually absent from the tissues of a calf with mannosidosis and the residual activity is due to component C. The acidic and neutral forms of α-mannosidase differ in their molecular weights and sensitivity to EDTA, Zn2+, Co2+ and Mn2+. An acidic α-mannosidase component (pH optimum 4.0) accounts for most of the activity in normal plasma but it is absent from the plasma of a calf with mannosidosis. Although the acidic α-mannosidase component is probably related to tissue components A and B, it can be distinguished from them by ion-exchange chromatography and gel filtration. The optimum pH of the low residual activity in the plasma from a calf with mannosidosis is pH5.5–5.75. The results support the hypothesis that Angus-cattle mannosidosis is a storage disease caused by a deficiency of lysosomal acidic α-mannosidase activity.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. CONCHIE J., MANN T. Glycosidases in mammalian sperm and seminal plasma. Nature. 1957 Jun 8;179(4571):1190–1191. doi: 10.1038/1791190a0. [DOI] [PubMed] [Google Scholar]
  2. Carroll M., Dance N., Masson P. K., Robinson D., Winchester B. G. Human mannosidosis--the enzyme defect. Biochem Biophys Res Commun. 1972 Oct 17;49(2):579–583. doi: 10.1016/0006-291x(72)90450-0. [DOI] [PubMed] [Google Scholar]
  3. Courtois J. E., Mangeot M. Sur quelques caractéres de l'alpha-mannosidase du sérum. Son activation par les sels de cobalt. Ann Biol Clin (Paris) 1972;30(1):49–58. [PubMed] [Google Scholar]
  4. HERS H. G. INBORN LYSOSOMAL DISEASES. Gastroenterology. 1965 May;48:625–633. [PubMed] [Google Scholar]
  5. Hocking J. D., Jolly R. D., Batt R. D. Deficiency of alpha-mannosidase in Angus cattle. An inherited lysosomal storage disease. Biochem J. 1972 Jun;128(1):69–78. doi: 10.1042/bj1280069. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Hultberg B. Properties of alpha-mannosidase in mannosidosis. Scand J Clin Lab Invest. 1970 Sep;26(2):155–159. doi: 10.3109/00365517009049228. [DOI] [PubMed] [Google Scholar]
  7. Jolly R. D. The pathology of the central nervous system in pseudolipidosis of Angus calves. J Pathol. 1971 Feb;103(2):113–121. doi: 10.1002/path.1711030206. [DOI] [PubMed] [Google Scholar]
  8. Kjellman B., Gamstorp I., Brun A., Ockerman P. A., Palmgren B. Mannosidosis: a clinical and histopathologic study. J Pediatr. 1969 Sep;75(3):366–373. doi: 10.1016/s0022-3476(69)80260-x. [DOI] [PubMed] [Google Scholar]
  9. Langley T. J., Jevons F. R. Characterization of beef-liver glycosidases possibly involved in glycoprotein degradation. Arch Biochem Biophys. 1968 Nov;128(2):312–318. doi: 10.1016/0003-9861(68)90037-4. [DOI] [PubMed] [Google Scholar]
  10. Marsh C. A., Gourlay G. C. Evidence for a non-lysosomal alpha-mannosidase in rat liver homogenates. Biochim Biophys Acta. 1971 Apr 14;235(1):142–148. doi: 10.1016/0005-2744(71)90041-6. [DOI] [PubMed] [Google Scholar]
  11. Mellors A., Harwalkar V. R. Glycosidases in bovine milk: alpha-mannosidase and its inhibition by zwitterions. Can J Biochem. 1968 Nov;46(11):1351–1356. doi: 10.1139/o68-205. [DOI] [PubMed] [Google Scholar]
  12. Nordén N. E., Lundblad A., Ockerman P. A., Jolly R. D. Mannosidosis in Angus cattle: partial characterization of two mannose containing oligosaccharides. FEBS Lett. 1973 Sep 15;35(2):209–212. doi: 10.1016/0014-5793(73)80286-8. [DOI] [PubMed] [Google Scholar]
  13. Nordén N. E., Lundblad A., Svensson S., Ockerman P. A., Autio S. A mannose-containing trisaccharide isolated from urines of three patients with mannosidosis. J Biol Chem. 1973 Sep 10;248(17):6210–6215. [PubMed] [Google Scholar]
  14. Ockerman P. A. Fluorimetric estimation of 4-methyl-umbelliferyl-alpha-mannosidase activity in blood plasma. Clin Chim Acta. 1969 Mar;23(3):479–482. doi: 10.1016/0009-8981(69)90353-2. [DOI] [PubMed] [Google Scholar]
  15. Ockerman P. A. Mannosidosis: isolation of oligosaccharide storage material from brain. J Pediatr. 1969 Sep;75(3):360–365. doi: 10.1016/s0022-3476(69)80259-3. [DOI] [PubMed] [Google Scholar]
  16. Poenaru L., Dreyfus J. C. Electrophoretic heterogeneity of human -mannosidase. Biochim Biophys Acta. 1973 Mar 23;303(1):171–174. doi: 10.1016/0005-2795(73)90158-x. [DOI] [PubMed] [Google Scholar]
  17. Robinson D., Price R. G., Dance N. Separation and properties of beta-galactosidase, beta-glucosidase, beta-glucuronidase and N-acetyl-beta-glucosaminidase from rat kidney. Biochem J. 1967 Feb;102(2):525–532. doi: 10.1042/bj1020525. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. SMITHIES O. Zone electrophoresis in starch gels: group variations in the serum proteins of normal human adults. Biochem J. 1955 Dec;61(4):629–641. doi: 10.1042/bj0610629. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Snaith S. M., Levvy G. A. Purification and properties of alpha-D-mannosidase from rat epididymis. Biochem J. 1969 Aug;114(1):25–33. doi: 10.1042/bj1140025. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Suzuki I., Kushida H., Shida H. [Mammalian glycosidases. IV. Characterization of alpha-D-mannosidase, alpha-D-glucosidase and beta-D-galactosidase and other various glycosidase activities in organs of rabbits and cats]. Seikagaku. 1970 Jul;42(7):361–371. [PubMed] [Google Scholar]

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