Ceramides as novel disease biomarkers

J Kurz, MJ Parnham, G Geisslinger… - Trends in molecular …, 2019 - cell.com
Ceramides are sphingolipids and integral components of the eukaryotic cell membrane.
Apart from providing structural integrity, ceramides have also been shown to act as second …

Acid ceramidase deficiency: Farber disease and SMA-PME

FPS Yu, S Amintas, T Levade, JA Medin - Orphanet journal of rare …, 2018 - Springer
Acid ceramidase (ACDase) deficiency is a spectrum of disorders that includes a rare
lysosomal storage disorder called Farber disease (FD) and a rare epileptic disorder called …

Sphingolipids contribute to human atherosclerotic plaque inflammation

A Edsfeldt, P Dunér, M Ståhlman, IG Mollet… - … , and vascular biology, 2016 - Am Heart Assoc
Objective—Lipids are central to the development of atherosclerotic plaques. Specifically,
which lipids are culprits remains controversial, and promising targets have failed in clinical …

Gene therapy for lysosomal storage diseases: Current clinical trial prospects

J Kido, K Sugawara, K Nakamura - Frontiers in genetics, 2023 - frontiersin.org
Lysosomal storage diseases (LSDs) are a group of metabolic inborn errors caused by
defective enzymes in the lysosome, resulting in the accumulation of undegraded substrates …

Quality of life and a surveillant endocannabinoid system

RA de Melo Reis, AR Isaac, HR Freitas… - Frontiers in …, 2021 - frontiersin.org
The endocannabinoid system (ECS) is an important brain modulatory network. ECS
regulates brain homeostasis throughout development, from progenitor fate decision to neuro …

Current treatment options and novel nanotechnology-driven enzyme replacement strategies for lysosomal storage disorders

A Del Grosso, G Parlanti, R Mezzena… - Advanced Drug Delivery …, 2022 - Elsevier
Lysosomal storage disorders (LSDs) are a vast group of more than 50 clinically identified
metabolic diseases. They are singly rare, but they affect collectively 1 on 5,000 live births …

Genetic ablation of acid ceramidase in Krabbe disease confirms the psychosine hypothesis and identifies a new therapeutic target

Y Li, Y Xu, BA Benitez, MS Nagree… - Proceedings of the …, 2019 - National Acad Sciences
Infantile globoid cell leukodystrophy (GLD, Krabbe disease) is a fatal demyelinating disorder
caused by a deficiency in the lysosomal enzyme galactosylceramidase (GALC). GALC …

The ceramide system as a novel antidepressant target

J Kornhuber, CP Müller, KA Becker, M Reichel… - Trends in …, 2014 - cell.com
Major depression is a systems disorder which impairs not only central nervous system
aspects of mood and behavior but also peripheral organ systems. Current views on the …

Sphingolipid lysosomal storage diseases: from bench to bedside

M Abed Rabbo, Y Khodour, LS Kaguni… - Lipids in health and …, 2021 - Springer
Abstract Johann Ludwig Wilhelm Thudicum described sphingolipids (SLs) in the late
nineteenth century, but it was only in the past fifty years that SL research surged in …

Structural basis for the activation of acid ceramidase

A Gebai, A Gorelik, Z Li, K Illes, B Nagar - Nature communications, 2018 - nature.com
Abstract Acid ceramidase (aCDase, ASAH1) hydrolyzes lysosomal membrane ceramide into
sphingosine, the backbone of all sphingolipids, to regulate many cellular processes …