Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency.

[1]  M. Vanier,et al.  Induced mouse models of abnormal sphingolipid metabolism. , 1998, Journal of biochemistry.

[2]  Michael P. McDonald,et al.  Mice lacking both subunits of lysosomal β–hexosaminidase display gangliosidosis and mucopolysaccharidosis , 1996, Nature Genetics.

[3]  A. Futerman,et al.  The localization of gangliosides in neurons of the central nervous system: the use of anti-ganglioside antibodies. , 1996, Biochimica et biophysica acta.

[4]  K. Livak,et al.  Real time quantitative PCR. , 1996, Genome research.

[5]  C. Heid,et al.  A novel method for real time quantitative RT-PCR. , 1996, Genome research.

[6]  N. Maeda,et al.  Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids. , 1996, Human molecular genetics.

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

[8]  Michael P. McDonald,et al.  Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism , 1995, Nature Genetics.

[9]  M. Baumann,et al.  Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL). , 1995, American journal of medical genetics.

[10]  V. Gieselmann,et al.  Lysosomal storage diseases. , 1995, Biochimica et biophysica acta.

[11]  T. Beccari,et al.  GM2 activator protein expression in mouse tissues. , 1994, Biochemical and biophysical research communications.

[12]  A. Poulos,et al.  Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene. , 1992, The Journal of biological chemistry.

[13]  J. Walker,et al.  Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease). , 1992, American journal of medical genetics.

[14]  K. Sandhoff,et al.  Modulation of Ganglioside Biosynthesis in Primary Cultured Neurons , 1989, Journal of neurochemistry.

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