Abstract
The skeletal muscle of cattle suffering from generalized glycogenosis type II was shown to lack acid α-glucosidase (EC 3.2.1.3) activity. Furthermore, there was no evidence of enzymically inactive proteins that cross-reacted with antibodies raised against acid α-glucosidase from the muscle of normal animals.
Full text
PDFSelected References
These references are in PubMed. This may not be the complete list of references from this article.
- BAUDHUIN P., HERS H. G., LOEB H. AN ELECTRON MICROSCOPIC AND BIOCHEMICAL STUDY OF TYPE II GLYCOGENOSIS. Lab Invest. 1964 Sep;13:1139–1152. [PubMed] [Google Scholar]
- Beratis N. G., LaBadie G. U., Hirschhorn K. Characterization of the molecular defect in infantile and adult acid alpha-glucosidase deficiency fibroblasts. J Clin Invest. 1978 Dec;62(6):1264–1274. doi: 10.1172/JCI109247. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Bruni C. B., Auricchio F., Covelli I. Acid alpha-D-glucosidase glucohydrolase from cattle liver. J Biol Chem. 1969 Sep 10;244(17):4735–4742. [PubMed] [Google Scholar]
- Burditt L. J., Chotai K., Hirani S., Nugent P. G., Winchester B. G., Blakemore W. F. Biochemical studies on a case of feline mannosidosis. Biochem J. 1980 Sep 1;189(3):467–473. doi: 10.1042/bj1890467. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Burditt L. J., Phillips N. C., Robinson D., Winchester B. G., Van-de-Water N. S., Jolly R. D. Characterization of the mutant alpha-mannosidase in bovine mannosidosis. Biochem J. 1978 Dec 1;175(3):1013–1022. doi: 10.1042/bj1751013. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Desnick R. J., Thorpe S. R., Fiddler M. B. Toward enzyme therapy for lysosomal storage diseases. Physiol Rev. 1976 Jan;56(1):57–99. doi: 10.1152/physrev.1976.56.1.57. [DOI] [PubMed] [Google Scholar]
- Dreyfus J. C., Proux D., Alexandre Y. Molecular studies on glycogen storage diseases. Enzyme. 1974;18(1):60–72. doi: 10.1159/000459414. [DOI] [PubMed] [Google Scholar]
- HERS H. G. INBORN LYSOSOMAL DISEASES. Gastroenterology. 1965 May;48:625–633. [PubMed] [Google Scholar]
- HERS H. G. alpha-Glucosidase deficiency in generalized glycogenstorage disease (Pompe's disease). Biochem J. 1963 Jan;86:11–16. doi: 10.1042/bj0860011. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Jolly R. D., Van-de-Water N. S., Richards R. B., Dorling P. R. Generalized glycogenosis in beef shorthorn cattle--heterozygote detection. Aust J Exp Biol Med Sci. 1977 Apr;55(2):14U–150. [PubMed] [Google Scholar]
- Murray A. K., Brown B. I., Brown D. H. The molecular heterogeneity of purified human liver lysosomal alpha-glucosidase (acid alpha-glucosidase). Arch Biochem Biophys. 1978 Jan 30;185(2):511–524. doi: 10.1016/0003-9861(78)90196-0. [DOI] [PubMed] [Google Scholar]
- Phillips N. C., Robinson D., Winchester B. G. Characterization of human liver alpha-D-mannosidase purified by affinity chromatography. Biochem J. 1976 Mar 1;153(3):579–587. doi: 10.1042/bj1530579. [DOI] [PMC free article] [PubMed] [Google Scholar]
- de Barsy T., Jacquemin P., Devos P., Hers H. G. Rodent and human acid -glucosidase. Purification, properties and inhibition by antibodies. Investigation in type II glycogenosis. Eur J Biochem. 1972 Nov 21;31(1):156–165. doi: 10.1111/j.1432-1033.1972.tb02514.x. [DOI] [PubMed] [Google Scholar]