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Interspecific genetic complementation analysis of human and sheep fibroblasts with beta-galactosidase deficiency


, : Interspecific genetic complementation analysis of human and sheep fibroblasts with beta-galactosidase deficiency. Somatic Cell and Molecular Genetics 15(6): 525-533

Interspecific somatic cell hybrids were analyzed by genetic complementation to determine if a lysosomal storage disease in sheep associated with deficiencies of beta-galactosidase and alpha-neuraminidase was homologous with any of four beta-galactosidase-deficient human diseases. Fibroblasts from beta-galactosidase-deficient sheep, cats, and human patients were fused and assayed histochemically for beta-galactosidase, with 5-bromo-4-chloro-3-indolyl beta-D-galactoside. We observed complementation in heterokaryons consisting of fibroblasts from beta-galactosidase-deficient sheep and fibroblasts from patients with galactosialidosis or mucolipidosis type II, but no complementation in heterokaryons consisting of fibroblasts from beta-galactosidase-deficient sheep and fibroblasts from human or feline GM1 gangliosidosis (type I) or from human mucopolysaccharidosis type IVB fibroblasts. We conclude that the ovine disease is due to a mutation at the genetic locus homologous with that of GM1 gangliosidosis and mucopolysaccharidosis type IVB, suggesting that the primary defect in the ovine disease is a mutation of the beta-galactosidase structural gene.

US$29.90

PMID: 2512653


Other references

Marshall, J.A., 1970: Photochemically Induced Ionic Reactions of Cycloalkenes: Cyclohexenes and cycloheptenes undergo cationic reactions upon ultraviolet irradiation in protic solvents. Sensitized ultraviolet irradiation of cyclohexenes and cycloheptenes in protic solvents causes reactions which seemingly proceed by way of carbocation intermediates. These intermediates, in turn, may arise from a photochemically produced trans cyc...

Goodarzi Fariborz; Cameron Alexander R., 1987: Distribution of major, minor and trace elements in coals of the Kootenay Group, Mount Allan, Alberta. The Canadian Mineralogist 25, Part 3(3): 555-565

Gusterson, F.R., 2012: Anaesthetic apparatus and cross-infection. British Medical Journal 1(4498): 388-388

Brainerd, C.J.; Allen, T.W., 1971: Experimental inductions of the conservation of "first-order" quantitative invariants. Psychological Bulletin 75(2): 128-144

Hegarty, M.; Yadav, R.; Lee, M.; Armstead, I.; Sanderson, R.; Scollan, N.; Powell, W.; Skøt, L., 2014: Genotyping by RAD sequencing enables mapping of fatty acid composition traits in perennial ryegrass (Lolium perenne (L.)). Perennial ryegrass (Lolium perenne L.) is the most important forage crop in temperate livestock agriculture. Its nutritional quality has significant impact on the quality of meat and milk for human consumption. Evidence suggests that higher energy...

Fontecilla Camps J.C.; Almassy R.J.; Suddath F.J.; Watt D.D.; Bugg C.E., 1980: 3 dimensional structure of a protein from scorpion venom a new structural class of neuro toxins. The 3-dimensional crystal structure of variant-3, toxin from the scorpion Centruroides sculpturatus was determined at 3 .ANG. resolution. Phases were obtained by use of K2PtCL4 and K2IrCl6 derivatives. The most prominent secondary structural featu...

Hirdes, J., P.; Maxwell, C., J., 1994: Smoking cessation and quality of life outcomes among older adults in the Campbell's survey on well-being. Most research regarding the health effects of smoking has focussed on mortality and on relatively young populations. Less is known about the consequences of smoking and the benefits of cessation among older adults. Improvements in quality of life...

Manegold, D., 1974: Agricultural income accounts in the member countries of the European Economic Community. Agrarwirtschaft 23 (3) 100-112

Cogan, D.C.; Kubik, C.S.; Smith, W.L., 1950: Unilateral internuclear ophthalmoplegia; a report of seven clinical cases and the postmortem findings in one case. Transactions of the American Neurological Association 51: 221-225

Pozsgay, Vince, 2001: Large scale synthesis of 2-azidodeoxy glucosyl donors. Starting from readily available phenyl 1-thio-beta-D-mannopyranoside, phenyl 2-azido-2-deoxy-1-thio-beta-D-glucopyranoside (9) was synthesized on multigram scale using inexpensive reagents.