Inhibition of fatty acid amide hydrolase prevents pathology in neurovisceral acid sphingomyelinase deficiency by rescuing defective endocannabinoid signaling
Abstract Acid sphingomyelinase deficiency (ASMD) leads to cellular accumulation of sphingomyelin (SM), neurodegeneration, and early death. Here, we describe the downregulation of the endocannabinoid (eCB) system in neurons of ASM knockout (ASM‐KO) mice and a ASMD patient. High SM reduced expression...
Saved in:
| Main Authors: | Adrián Bartoll, Ana Toledano‐Zaragoza, Josefina Casas, Manuel Guzmán, Edward H Schuchman, María Dolores Ledesma |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Springer Nature
2020-10-01
|
| Series: | EMBO Molecular Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.15252/emmm.201911776 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
Similar Items
-
Case report of the perinatal form of Nieman-Pick disease type С against the backdrop of pathogenetic off-label therapy
by: Nailya R. Mingacheva, et al.
Published: (2024-05-01) -
Niemann-Pick Disease, Type A: Clinical Case of 5 Months Old Patient
by: Nataliya V. Zhurkova, et al.
Published: (2024-07-01) -
Inability of α-cyclodextrins to accommodate cholesterol potentially underlies their lack of efficacy and ototoxicity in Niemann-Pick disease type C treatment
by: Yusei Yamada, et al.
Published: (2025-08-01) -
c-Abl/TFEB Pathway Activation as a Common Pathogenic Mechanism in Lysosomal Storage Diseases: Therapeutic Potential of c-Abl Inhibitors
by: Miguel V. Guerra, et al.
Published: (2025-05-01) -
A preliminary investigation of tobacco co-use on endocannabinoid activity in people with cannabis use
by: Rachel A. Rabin, et al.
Published: (2025-09-01)