Recombinant GM2-Activator Protein Stimulates In Vivo Degradation of GA2 in GM2 Gangliosidosis AB Variant Fibroblasts But Exhibits No Detectable Binding of GA2 in an In Vitro Assay

[1]  T. Kyrklund.  Two procedures to remove polar contaminants from a crude brain lipid extract by using prepacked reversed-phase columns , 1987, Lipids.

[2]  N. Kasai,et al.  A convenient method for the preparation of asialo-GM1 , 1982, Lipids.

[3]  W. Roggendorf,et al.  Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs , 1993, Human Genetics.

[4]  J. Crawley,et al.  Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment. , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[5]  Su-Chen Li,et al.  Interaction of GM2 Activator Protein with Glycosphingolipids* , 1997, The Journal of Biological Chemistry.

[6]  Y. Li,et al.  Interaction of GM2 activator protein with glycosphingolipids. , 1997, The Journal of biological chemistry.

[7]  U. Schepers,et al.  Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant. , 1996, American journal of human genetics.

[8]  K. Sandhoff,et al.  Topology of glycosphingolipid degradation. , 1996, Trends in cell biology.

[9]  K. Suzuki,et al.  Sphingolipid activator proteins. , 1995, Essays in biochemistry.

[10]  N. V. Pavlova,et al.  Expression and specificity of human GM2 activator protein. , 1994, The Journal of biological chemistry.

[11]  K. Sandhoff,et al.  Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo. , 1994, Biochemical and biophysical research communications.

[12]  K. Sandhoff,et al.  Chapter 2 Ganglioside metabolism: enzymology, topology and regulation , 1994 .

[13]  K. Sandhoff,et al.  Over-expression of a functionally active human GM2-activator protein in Escherichia coli. , 1993, The Biochemical journal.

[14]  K. Sandhoff,et al.  Ganglioside metabolism--topology and regulation. , 1993, Advances in lipid research.

[15]  A. Poulos,et al.  Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase. , 1992, The Biochemical journal.

[16]  K. Sandhoff,et al.  Activator proteins and topology of lysosomal sphingolipid catabolism. , 1992, Biochimica et biophysica acta.

[17]  A. M. Lawson,et al.  Specificity of lung surfactant protein SP-A for both the carbohydrate and the lipid moieties of certain neutral glycolipids. , 1992, The Journal of biological chemistry.

[18]  G. Schwarzmann,et al.  The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A. , 1991, The Journal of biological chemistry.

[19]  W. Machleidt,et al.  The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein. , 1990, European journal of biochemistry.

[20]  K. Sandhoff,et al.  Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A. , 1985, The Journal of biological chemistry.

[21]  G. Schwarzmann,et al.  Incorporation and metabolism of ganglioside GM2 in skin fibroblasts from normal and GM2 gangliosidosis subjects. , 1985, European journal of biochemistry.

[22]  K. Nakamura,et al.  Effect of modification of sialic acid on enzymic hydrolysis of gangliosides GM1 and GM2. , 1984, The Journal of biological chemistry.

[23]  K. Sandhoff,et al.  Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2. , 1982, European journal of biochemistry.

[24]  R. Yu,et al.  Gangliosides: structure, isolation, and analysis. , 1982, Methods in enzymology.

[25]  Y. Hirabayashi,et al.  A protein activator for the enzymic hydrolysis of GM2 ganglioside. , 1981, The Journal of biological chemistry.

[26]  K. Sandhoff,et al.  Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A. , 1979, Hoppe-Seyler's Zeitschrift fur physiologische Chemie.

[27]  K. Sandhoff,et al.  ENZYME ALTERATIONS AND LIPID STORAGE IN THREE VARIANTS OF TAY‐SACHS DISEASE , 1971, Journal of neurochemistry.