Acid Ceramidase Depletion Impairs Neuronal Survival and Induces Morphological Defects in Neurites Associated with Altered Gene Transcription and Sphingolipid Content

Genetic and pharmacological inhibition of acid ceramidase prevents asymmetric cell division by neosis

(2019) Shai White-Gilbertson et al.   JOURNAL OF LIPID RESEARCH

Control of Lysosomal TRPML1 Channel Activity and Exosome Release by Acid Ceramidase in Mouse Podocytes

(2019) Guangbi Li et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Chemotherapy selection pressure alters sphingolipid composition and mitochondrial bioenergetics in resistant HL-60 cells

(2019) LI-PIN KAO et al.   JOURNAL OF LIPID RESEARCH

Hepatic pathology and altered gene transcription in a murine model of acid ceramidase deficiency

(2019) Fabian P. S. Yu et al.   LABORATORY INVESTIGATION

Pathological manifestations of Farber disease in a new mouse model

(2018) Nadine Beckmann et al.   BIOLOGICAL CHEMISTRY

Mechanisms of lysosomal positioning and movement

(2018) Birol Cabukusta et al.   TRAFFIC

Altered lysosome distribution is an early neuropathological event in neurological forms of Gaucher disease

(2017) Hila Zigdon et al.   FEBS LETTERS

Complete Acid Ceramidase ablation prevents cancer-initiating cell formation in melanoma cells

(2017) Michele Lai et al.   Scientific Reports

An ER-Associated Pathway Defines Endosomal Architecture for Controlled Cargo Transport

(2016) Marlieke L.M. Jongsma et al.   CELL

Endolysosomes Are the Principal Intracellular Sites of Acid Hydrolase Activity

(2016) Nicholas A. Bright et al.   CURRENT BIOLOGY

ASAH1 variant causing a mild SMA phenotype with no myoclonic epilepsy: a clinical, biochemical and molecular study

(2016) Massimiliano Filosto et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Mechanisms and functions of lysosome positioning

(2016) Jing Pu et al.   JOURNAL OF CELL SCIENCE

A molecular mechanism to regulate lysosome motility for lysosome positioning and tubulation

(2016) Xinran Li et al.   NATURE CELL BIOLOGY

Loss of ceramide synthase 2 activity, necessary for myelin biosynthesis, precedes tau pathology in the cortical pathogenesis of Alzheimer's disease

(2016) Timothy A. Couttas et al.   NEUROBIOLOGY OF AGING

NAD+ repletion improves mitochondrial and stem cell function and enhances life span in mice

(2016) H. Zhang et al.   SCIENCE

Obatoclax impairs lysosomal function to block autophagy in cisplatin-sensitive and -resistant esophageal cancer cells

(2016) Le Yu et al.   Oncotarget

Spinal muscular atrophy associated with progressive myoclonic epilepsy: A rare condition caused by mutations inASAH1

(2015) Guido Rubboli et al.   EPILEPSIA

Altered lysosomal positioning affects lysosomal functions in a cellular model of Huntington's disease

(2015) Christine Erie et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Autophagosome dynamics in neurodegeneration at a glance

(2015) Y. C. Wong et al.   JOURNAL OF CELL SCIENCE

Acid ceramidase deficiency associated with spinal muscular atrophy with progressive myoclonic epilepsy

(2015) Joanna J. Gan et al.   NEUROMUSCULAR DISORDERS

Uniparental disomy as a cause of spinal muscular atrophy and progressive myoclonic epilepsy: Phenotypic homogeneity due to the homozygous c.125C>T mutation in ASAH1

(2015) Beatriz G. Giráldez et al.   NEUROMUSCULAR DISORDERS

Endolysosomal Deficits Augment Mitochondria Pathology in Spinal Motor Neurons of Asymptomatic fALS Mice

(2015) Yuxiang Xie et al.   NEURON

Massive accumulation of luminal protease-deficient axonal lysosomes at Alzheimer’s disease amyloid plaques

(2015) Swetha Gowrishankar et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Impairment of ceramide synthesis causes a novel progressive myoclonus epilepsy

(2014) Nicola Vanni et al.   ANNALS OF NEUROLOGY

Neurodegenerative lysosomal disorders: A continuum from development to late age

(2014) Ralph A. Nixon et al.   Autophagy

Rho family GTPases: key players in neuronal development, neuronal survival, and neurodegeneration

(2014) Trisha R. Stankiewicz et al.   Frontiers in Cellular Neuroscience

Reduced ceramide synthase 2 activity causes progressive myoclonic epilepsy

(2014) Mai-Britt Mosbech et al.   Annals of Clinical and Translational Neurology

Loss of Function of Glucocerebrosidase GBA2 Is Responsible for Motor Neuron Defects in Hereditary Spastic Paraplegia

(2013) Elodie Martin et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Evidence for clinical, genetic and biochemical variability in spinal muscular atrophy with progressive myoclonic epilepsy

(2013) D.A. Dyment et al.   CLINICAL GENETICS

Farber disease: understanding a fatal childhood disorder and dissecting ceramide biology

(2013) Mark S. Sands   EMBO Molecular Medicine

Systemic ceramide accumulation leads to severe and varied pathological consequences

(2013) Abdulfatah M. Alayoubi et al.   EMBO Molecular Medicine

Spinal Muscular Atrophy Associated with Progressive Myoclonic Epilepsy Is Caused by Mutations in ASAH1

(2012) Jie Zhou et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Cell cycle regulation of Rho signaling pathways

(2012) Muriel DAVID et al.   CELL CYCLE

Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments

(2012) Kristiina Uusi-Rauva et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Acid Ceramidase (ASAH1) Is a Global Regulator of Steroidogenic Capacity and Adrenocortical Gene Expression

(2012) Natasha C. Lucki et al.   MOLECULAR ENDOCRINOLOGY

The histone demethylase PHF8 is essential for cytoskeleton dynamics

(2012) Elena Asensio-Juan et al.   NUCLEIC ACIDS RESEARCH

Cancer-associated lysosomal changes: friends or foes?

(2012) T Kallunki et al.   ONCOGENE

The spinal muscular atrophy disease protein SMN is linked to the rho-kinase pathway via profilin

(2011) Anna Nölle et al.   HUMAN MOLECULAR GENETICS

Roles of the Drosophila LRRK2 homolog in Rab7-dependent lysosomal positioning

(2011) M. W. Dodson et al.   HUMAN MOLECULAR GENETICS

Lysosomal positioning coordinates cellular nutrient responses

(2011) Viktor I. Korolchuk et al.   NATURE CELL BIOLOGY

A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease

(2010) Carmen Bedia et al.   JOURNAL OF LIPID RESEARCH

Ceramide acyl chain length markedly influences miscibility with palmitoyl sphingomyelin in bilayer membranes

(2009) Bodil Westerlund et al.   EUROPEAN BIOPHYSICS JOURNAL WITH BIOPHYSICS LETTERS

Ceramide and neurodegeneration: Susceptibility of neurons and oligodendrocytes to cell damage and death

(2009) Arundhati Jana et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Ceramidases: regulators of cellular responses mediated by ceramide, sphingosine, and sphingosine-1-phosphate

(2008) C MAO   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Membrane Domain Formation, Interdigitation, and Morphological Alterations Induced by the Very Long Chain Asymmetric C24:1 Ceramide

(2008) Sandra N. Pinto et al.   BIOPHYSICAL JOURNAL

Autophagy in the Pathogenesis of Disease

(2008) Beth Levine et al.   CELL

Mammalian Rho GTPases: new insights into their functions from in vivo studies

(2008) Sarah J. Heasman et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Diverse roles of Rho family GTPases in neuronal development, survival, and death

(2007) Daniel et al.   Frontiers in Bioscience-Landmark