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Peripheral neuropathy in a mouse model lacking GBA1 in Schwann cells
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DOI：10.1016/j.nbd.2026.107562.png)
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Abstract
En 中文
• Schwann cell GBA1 deficiency alters myelination and recruits immune cell recruitment in adult peripheral nerves. • GBA1 deficient Schwann cells display a repair-like phenotype. • Schwann cell GBA1 KO results in adult-onset motor dysfunction. • Schwann cell dysfunction might contribute to the neuropathic phenotype detected in type I Gaucher disease.
Keywords:
β-glucocerebrosidase
Peripheral nervous system
Schwann cell
Myelination
Peripheral neuropathy
Myelinophagy
SC
Schwann Cell
GD
Gaucher Disease
PNS
Peripheral Nervous System
LSD
Lysosomal Storage Disorder
DMSO
Dimethyl sulfoxide
CBE
Conduritol-B-epoxide
dbcAMP
Dibutyryl-cAMP
SCCM
Schwann Cell Culture Medium
MUG
4-Methylumbelliferyl-β-D-glucuronide hydrate
TEM
Transmission Electron Microscopy
AChR
Acetylcholine Receptor
NMJ
Neuromuscular Junction
CMAP
Compound muscle action potential
NCV
Nerve conduction velocity
Journal
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5.6
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6.7K
Citations:
2.1W
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