Thus, neither of the two mouse models replicates the severe muscle phenotype and pathology seen in humans.
We have previously shown that primary-tumor derived orthotopic xenograft mouse models replicated the histology, invasive growth, and genetic abnormalities of pediatric brain tumors [ 45, 50].
The mouse models replicates the disease course in humans, clinical features and complications of SS and gives researchers insights into the disease pathogenesis.
Development of mouse models replicating mitochondrial disease phenotypes has provided a unique opportunity both for therapeutic trials and for detailed studies of molecular pathophysiology of primary mitochondrial dysfunction.
TGF-β expression and activity are increased in human RDEB and in mouse models replicating the disorder (Fritsch et al, 2008; Ng et al, 2012; Kuttner et al, 2013; Nystrom et al, 2013).
Since one pathological feature in MSA patients covers selective neuronal loss in the SNc, and the (PLP -αSYN mouse model rePLP -αSYNthis feature, we evaluated whether mousereatmodelhad an effect on numbereplicateseurons.
Both mouse-models replicate the NCL phenotype and neuropathology; the Cln1-/ model presents with early onset, severe neurodegenerative disease, whereas the Cln5-/ model produces a milder disease with a later onset.
A roadblock to the mechanistic understanding in part can be attributed to the inability of existing XP mouse models to replicate the neurological pathologies of XP patients (Nakane et al., 1995; Andressoo et al., 2009).
However, due to differences in the physiological traits and gene expression between mice and humans, mouse models cannot replicate the symptoms or pathology of human diseases in some cases [ 1].
