Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=29563343
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\29563343
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 JCI+Insight
2018 ; 3
(6
): ä Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Neuropathic pain in a Fabry disease rat model
#MMPMID29563343
Miller JJ
; Aoki K
; Moehring F
; Murphy CA
; O'Hara CL
; Tiemeyer M
; Stucky CL
; Dahms NM
JCI Insight
2018[Mar]; 3
(6
): ä PMID29563343
show ga
Fabry disease, the most common lysosomal storage disease, affects multiple organs
and results in a shortened life span. This disease is caused by a deficiency of
the lysosomal enzyme ?-galactosidase A, which leads to glycosphingolipid
accumulation in many cell types. Neuropathic pain is an early and severely
debilitating symptom in patients with Fabry disease, but the cellular and
molecular mechanisms that cause the pain are unknown. We generated a rat model of
Fabry disease, the first nonmouse model to our knowledge. Fabry rats had
substantial serum and tissue accumulation of ?-galactosyl glycosphingolipids and
had pronounced mechanical pain behavior. Additionally, Fabry rat dorsal root
ganglia displayed global N-glycan alterations, sensory neurons were laden with
inclusions, and sensory neuron somata exhibited prominent sensitization to
mechanical force. We found that the cation channel transient receptor potential
ankyrin 1 (TRPA1) is sensitized in Fabry rat sensory neurons and that TRPA1
antagonism reversed the behavioral mechanical sensitization. This study points
toward TRPA1 as a potentially novel target to treat the pain experienced by
patients with Fabry disease.
|Animals
[MESH]
|Animals, Genetically Modified
[MESH]
|Behavior, Animal
[MESH]
|Disease Models, Animal
[MESH]
|Electrophysiology
[MESH]
|Fabry Disease/*complications/*metabolism
[MESH]
|Female
[MESH]
|Ganglia, Spinal/metabolism/pathology
[MESH]
|Gene Knockdown Techniques
[MESH]
|Genetic Predisposition to Disease/genetics
[MESH]