Characterization of small fiber pathology in a mouse model of Fabry disease
- University of Würzburg, Germany
- Yale Medical School and Veterans Affairs Hospital, United States
Figures
Figure 1
Toluidin blue staining and immunoreaction against globotriaosylceramide and immunoglobulin binding protein of mouse dorsal root ganglia.
Photomicrographs display hematoxylin-eosin staining DRG neurons from young and old GLA KO and WT mice (A–D) and exemplified measured cell area (yellow circles). (E) Quantification of neuronal cell …
Figure 2
Reduced intraepidermal nerve fiber density in α-galactosidase A deficient mice and Gb3 distribution in sciatic nerve and skin.
Photomicrographs show immunoreactivity of antibodies against protein gene product 9.5 (PGP 9.5) as a pan-axonal marker in 40 µm skin sections from footpads of young (3 months) and old (≥12 months) …
Figure 3
More apoptosis and less neurite outgrowth in dorsal root ganglion neurons of old α-galactosidase A deficient mice compared to wildtype mice.
Photomicrographs show the results of a NucView 488 Caspase 3 Enzyme Substrate Assay of cultivated dorsal root ganglion (DRG) neurons from old (≥12 months) wildtype (WT) and α-galactosidase A …
Figure 4
Expression, function, and phenotypic reflection of transient receptor potential vanilloid one channels in α-galactosidase A deficient mice.
(A) Boxplots show the results of transient receptor potential vanilloid 1 (TRPV1) channel gene expression in dorsal root ganglia (DRG) of young (3 months) and old (≥12 months) wildtype (WT) and …
Figure 5
Expression, function, and phenotypic reflection of hyperpolarization-activated cyclic nucleotide-gated ion channels in α-galactosidase A deficient mice.
(A) Boxplots show the results of potassium/sodium hyperpolarization-activated cyclic nucleotide-gated ion channel 2 (HCN2) gene expression in dorsal root ganglia (DRG) of young (3 months) and old …
Figure 6
Expression, function, and phenotypic reflection of voltage-gated soium channel 1.7 in α-galactosidase A deficient mice.
(A) Boxplots show the results of voltage-gated sodium channel 1.7 (Nav1.7) gene expression in dorsal root ganglia (DRG) of young (3 months) and old (≥12 months) wildtype (WT) and α-galactosidase A …
Figure 7
Knock-down of α-galactosidase A in human embryonic kidney 293 cells expressing voltage-gated sodium channel 1.7.
Photomicrographs show immunoreactivity of antibodies against CD77 as a marker for globotriaosylceramide (Gb3) accumulation in human embryonic kidney 293 (HEK) cells expressing voltage-gated sodium …
Author response image 1
Mechanical sensitivity after injection of complete Freund`s adjuvant.
Line chart displays mechanical withdrawal thresholds from old (≥12 months) wildtype (WT) and α-galactosidase A deficient (GLA KO) mice, calculated as percent change from baseline. Old WT mice were …
Videos
Video 1
Localization of globotriaosylceramide in dorsal root ganglion neurons of an old α-galactosidase A deficient mouse
Video shows immunoreaction against CD77 (red) as a marker for globotriaosylceramide (Gb3) accumulation and β-(ΙΙΙ)-tubulin (green) as a neuron specific cytoplasm marker, in dorsal root ganglion …
Additional files
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Transparent reporting form
- https://doi.org/10.7554/eLife.39300.011
