Results 101 to 110 of about 4,601 (147)

Adults With Acid Sphingomyelinase Deficiency Have Sustained Improvements in Clinical Outcomes With up to 5 Years of Olipudase Alfa Enzyme Replacement Therapy: ASCEND Trial Final Results. [PDF]

open access: yesJ Inherit Metab Dis
Wasserstein MP   +20 more
europepmc   +1 more source

Early secretory pathway-resident Zn transporter proteins contribute to cellular sphingolipid metabolism through activation of sphingomyelin phosphodiesterase 1

open access: yesEarly secretory pathway-resident Zn transporter proteins contribute to cellular sphingolipid metabolism through activation of sphingomyelin phosphodiesterase 1
Sphingomyelin phosphodiesterase 1 (SMPD1) converts sphingomyelin into ceramide and phosphocholine; hence, loss of SMPD1 function causes abnormal accumulation of sphingomyelin in lysosomes, which results in the lipid-storage disorder Niemann-Pick disease (types A and B). SMPD1 activity is dependent on zinc, which is coordinated at the active site of the
openaire  

Bacterial extracellular vesicles as recyclable nutrient reservoirs. [PDF]

open access: yesNat Commun
Laimer-Digruber A   +13 more
europepmc   +1 more source

Metabolic improvement in patients with acid sphingomyelinase deficiency following intravenous trehalose administration: an untargeted pharmacometabolomic study. [PDF]

open access: yesOrphanet J Rare Dis
Khoshakhlagh M   +7 more
europepmc   +1 more source

Lipid metabolism as a marker for glioma aggressiveness. [PDF]

open access: yesBiosci Rep
Ribas HT   +9 more
europepmc   +1 more source

Molecular Maestro in the Lung: The Sphingolipid Rheostat. [PDF]

open access: yesAm J Respir Cell Mol Biol
Rojas-Quintero J, Polverino F.
europepmc   +1 more source

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