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  4. Blood Flow to the Spleen is Altered in a Mouse Model of Spinal Muscular Atrophy
 
research article

Blood Flow to the Spleen is Altered in a Mouse Model of Spinal Muscular Atrophy

Deguise, Marc-Olivier
•
Beauvais, Ariane
•
Schneider, Bernard L.  
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January 1, 2020
Journal Of Neuromuscular Diseases

Spinal muscular atrophy (SMA) is a neuromuscular disorder affecting young children. While pre-clinical models of SMA show small spleens, the same is not true in humans. Here, we show by doppler ultrasonography decreased splenic blood flow in Smn(2B/-) mice. Further, AAV9-SMN gene therapy does not rescue the distal ear and tail necrosis nor the spleen size in these mice, suggesting that the latter may be linked to a cardiovascular defect. Absence of smaller spleens in human patients is likely due to differences in presentation of defects in SMA between pre-clinical mouse models and human patients, particularly the susceptibility to cardiovascular issues.

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Type
research article
DOI
10.3233/JND-200493
Web of Science ID

WOS:000685106600012

Author(s)
Deguise, Marc-Olivier
Beauvais, Ariane
Schneider, Bernard L.  
Kothary, Rashmi
Date Issued

2020-01-01

Published in
Journal Of Neuromuscular Diseases
Volume

7

Issue

3

Start page

315

End page

322

Subjects

Clinical Neurology

•

Neurosciences

•

Neurosciences & Neurology

•

immune system

•

perfusion

•

blood flow

•

vasculature

•

mouse models

•

cardiac defects

•

restoration

•

phenotype

•

rescue

•

mice

Editorial or Peer reviewed

REVIEWED

Written at

EPFL

EPFL units
PTBTG  
Available on Infoscience
August 28, 2021
Use this identifier to reference this record
https://infoscience.epfl.ch/handle/20.500.14299/180965
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