PMID- 36875645
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230307
IS  - 1662-4548 (Print)
IS  - 1662-453X (Electronic)
IS  - 1662-453X (Linking)
VI  - 17
DP  - 2023
TI  - Sphingolipids in neurodegenerative diseases.
PG  - 1137893
LID - 10.3389/fnins.2023.1137893 [doi]
LID - 1137893
AB  - Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive 
      deficits of neuronal function. Recent evidence argues that sphingolipid 
      metabolism is affected in a surprisingly broad set of NDDs. These include some 
      lysosomal storage diseases (LSDs), hereditary sensory and autonomous neuropathy 
      (HSAN), hereditary spastic paraplegia (HSP), infantile neuroaxonal dystrophy 
      (INAD), Friedreich's ataxia (FRDA), as well as some forms of amyotrophic lateral 
      sclerosis (ALS) and Parkinson's disease (PD). Many of these diseases have been 
      modeled in Drosophila melanogaster and are associated with elevated levels of 
      ceramides. Similar changes have also been reported in vertebrate cells and mouse 
      models. Here, we summarize studies using fly models and/or patient samples which 
      demonstrate the nature of the defects in sphingolipid metabolism, the organelles 
      that are implicated, the cell types that are initially affected, and potential 
      therapeutics for these diseases.
CI  - Copyright (c) 2023 Pan, Dutta, Lu and Bellen.
FAU - Pan, Xueyang
AU  - Pan X
AD  - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, 
      TX, United States.
AD  - Jan and Dan Duncan Neurological Research Institute, Texas Children's Hospital, 
      Houston, TX, United States.
FAU - Dutta, Debdeep
AU  - Dutta D
AD  - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, 
      TX, United States.
AD  - Jan and Dan Duncan Neurological Research Institute, Texas Children's Hospital, 
      Houston, TX, United States.
FAU - Lu, Shenzhao
AU  - Lu S
AD  - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, 
      TX, United States.
AD  - Jan and Dan Duncan Neurological Research Institute, Texas Children's Hospital, 
      Houston, TX, United States.
FAU - Bellen, Hugo J
AU  - Bellen HJ
AD  - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, 
      TX, United States.
AD  - Jan and Dan Duncan Neurological Research Institute, Texas Children's Hospital, 
      Houston, TX, United States.
AD  - Department of Neuroscience, Baylor College of Medicine, Houston, TX, United 
      States.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20230216
PL  - Switzerland
TA  - Front Neurosci
JT  - Frontiers in neuroscience
JID - 101478481
PMC - PMC9978793
OTO - NOTNLM
OT  - Drosophila
OT  - Parkinson's disease
OT  - ceramides
OT  - lysosome
OT  - mitochondria
OT  - neurodegeneration
OT  - sphingolipids
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/03/07 06:00
MHDA- 2023/03/07 06:01
PMCR- 2023/01/01
CRDT- 2023/03/06 04:12
PHST- 2023/01/05 00:00 [received]
PHST- 2023/01/27 00:00 [accepted]
PHST- 2023/03/06 04:12 [entrez]
PHST- 2023/03/07 06:00 [pubmed]
PHST- 2023/03/07 06:01 [medline]
PHST- 2023/01/01 00:00 [pmc-release]
AID - 10.3389/fnins.2023.1137893 [doi]
PST - epublish
SO  - Front Neurosci. 2023 Feb 16;17:1137893. doi: 10.3389/fnins.2023.1137893. 
      eCollection 2023.