Several reports have described magnetic resonance (MR) findings in canine and feline lysosomal storage diseases such as gangliosidoses and neuronal ceroid lipofuscinosis. Although most of those studies described the signal intensities of white matter in the cerebrum, findings of the corpus callosum were not described in detail. A retrospective study was conducted on MR findings of the corpus ca...

To investigate whether antecedent cytomegalovirus (CMV) infections in patients with Guillain-Barre syndrome are associated with the presence of specific antiganglioside antibodies, acute phase serum samples from 130 patients with Guillain-Barre syndrome and 200 controls were tested. Anti-GM2 IgM antibodies were found more often in patients with Guillain-Barre syndrome with CMV infection (22%) t...

The level of the neutral glycolipid, Galnac beta 1 leads to 4 Gal beta 1 leads to 4Glc leads to Cer (asialo GM2), in BALB/c 3T3 mouse fibroblasts transformed by Kirsten murine sarcoma virus (3T3KIMSV) was greatly increased compared to the nontransformed parental cells (3T3). This elevated chemical quantity was found to be localized on the surface of intact cells and accessible to external reage...

Sandhoff Disease (SD) is an autosomal recessive neurodegenerative disease caused by a mutation in the Hexb gene for the β-subunit of β-hexosaminidase A, resulting in the inability to catabolize ganglioside GM2 within the lysosomes. SD presents with an accumulation of GM2 and its asialo derivative GA2 primarily in the CNS. Myelin-enriched glycolipids, cerebrosides and sulfatides, are also decrea...

synthesis and characterization of 6-(hydroxymethyl)-14, 16-dimethyl-13, 14, 16, 17-tetrahydro-6h-13, 17-epiminodibenzo [e, l] [1, 4] dioxacyclotridecin-15 (7h)-oneabstractnew 6-(hydroxymethyl)-14, 16-dimethyl-13, 14, 16, 17-tetrahydro-6h-13, 17-epiminodibenzo [e, l] [1, 4] dioxacyclotridecin-15 (7h)-one (2a) was synthesized in good yield by the petrenko–kritchenko reaction of 2, 2'-((3-hydroxyp...

Juvenile GM2 gangliosidosis (jGM2) is a group of inherited neurodegenerative diseases caused by deficiency of lysosomal β-hexosaminidase A (Hex A) resulting in GM2 ganglioside accumulation in brain. Like many other lysosomal storage diseases (LSDs), no specific treatment currently exists. In order to establish clinical outcomes for the investigation of potential therapies for jGM2, I collected ...