… the neurodegenerative murine LSD models available that recapitulate human disease, and
… some limitations and difficulties in working with mouse models of neurodegenerative LSDs. …

… For each disorder, the causative gene has been identified … and astrocytes of a Gaucher
disease mouse model [119]. This … can still hold value as markers of disease progression. Of note, …

… that may complicate phenotype identification. Models of neurodegenerative diseases using
… Developmental studies in animal models have greatly accelerated our understanding of the …

… The use of sphingolipids as new diagnostic markers and as … There is no reasonable
transgenic animal model which would … More detailed characteristics of rafts, identified at different …

… The [mH]− ions of G M2 and G M3 were used to identify the gangliosides with the N-…
male MPS IIIA mice exhibited significant elevations in the two markers of neuroinflammation …

… We also identify parallels between the sphingolipidoses and … An increase in inflammatory
markers correlated with disease … mice, a naturally occurring mouse model of Krabbe disease, …

… of 36 identified proteins were related to neurodegeneration. … studies in cellular and animal
models of NPC disease. Most of … MPS VII (also known as Sly disease) is a LSD that caused by …

… , end detection was performed via a third fluorescent marker, … in neurodegeneration to help
understand the basis of disease … Moreover, using a mouse model of Niemann–Pick disease …

… MPS type VII (OMIM #253220), or Sly syndrome, is an … other neurodegenerative disease
in which iPSC-based models are … on cell models, the unquestionable role that animal models …

… cellular models in drug discovery processes aimed at identifying … recently provided in mouse
models of Sly disease and MLD. … to identify membrane-bound cell-specific markers could …