The cell biology of lysosomal storage disorders

AH Futerman, G Van Meer - Nature reviews Molecular cell biology, 2004 - nature.com
Lysosomal storage disorders, of which more than 40 are known, are caused by the
defective activity of lysosomal proteins, which results in the intra-lysosomal accumulation of …

The complex life of simple sphingolipids

AH Futerman, YA Hannun - EMBO reports, 2004 - embopress.org
The extensive diversity of membrane lipids is rarely appreciated by cell and molecular
biologists. Although most researchers are familiar with the three main classes of lipids in animal …

Mammalian ceramide synthases

M Levy, AH Futerman - IUBMB life, 2010 - Wiley Online Library
… Work in the Futerman laboratory on the CerS proteins is supported by the Israel Science
Foundation (1404/07), the National Institutes of Health (GM076217), the Minerva Foundation, …

[HTML][HTML] Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate

…, S Epstein, H Park, AH Merrill, AH Futerman - Journal of Biological …, 2008 - ASBMB
Ceramide is an important lipid signaling molecule and a key intermediate in sphingolipid
biosynthesis. Recent studies have implied a previously unappreciated role for the ceramide N-…

[HTML][HTML] When do Lasses (longevity assurance genes) become CerS (ceramide synthases)?: Insights into the regulation of ceramide synthesis

Y Pewzner-Jung, S Ben-Dor, AH Futerman - Journal of Biological Chemistry, 2006 - ASBMB
Ceramide is the key intermediate in the pathway of sphingolipid (SL) 2 biosynthesis (1) and
an important intracellular signaling molecule (2, 3). Ceramide consists of a sphingoid long …

[HTML][HTML] Two mammalian longevity assurance gene (LAG1) family members, trh1 and trh4, regulate dihydroceramide synthesis using different fatty acyl-CoA donors

…, JC Allegood, E Wang, AH Merrill, AH Futerman - Journal of Biological …, 2003 - ASBMB
Overexpression of upstream of growth and differentiation factor 1 (uog1), a mammalian
homolog of the yeast longevity assurance gene (LAG1), selectively induces the synthesis of …

[PDF][PDF] CerS2 haploinsufficiency inhibits β-oxidation and confers susceptibility to diet-induced steatohepatitis and insulin resistance

…, K Takeda, S Sugii, Y Pewzner-Jung, AH Futerman… - Cell metabolism, 2014 - cell.com
Inhibition of ceramide synthesis prevents diabetes, steatosis, and cardiovascular disease in
rodents. Six different ceramide synthases (CerS) that differ in tissue distribution and …

Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system

…, I Silman, JL Sussman, AH Futerman… - Plant biotechnology …, 2007 - Wiley Online Library
Gaucher's disease, a lysosomal storage disorder caused by mutations in the gene encoding
glucocerebrosidase (GCD), is currently treated by enzyme replacement therapy using …

[HTML][HTML] Common and uncommon pathogenic cascades in lysosomal storage diseases

EB Vitner, FM Platt, AH Futerman - Journal of Biological Chemistry, 2010 - ASBMB
Lysosomal storage diseases (LSDs), of which about 50 are known, are caused by the defective
activity of lysosomal proteins, resulting in accumulation of unmetabolized substrates. As …

The ins and outs of sphingolipid synthesis

AH Futerman, H Riezman - Trends in cell biology, 2005 - cell.com
Sphingolipids are ubiquitous components of eukaryotic cell membranes, where they play
important roles in intracellular signaling and in membrane structure. Even though the …