Results 41 to 50 of about 1,124 (173)

A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A

open access: yesJournal of Lipid Research, 2005
A mass spectrometric method is described for monitoring cerebrosides in the presence of excess concentrations of alkali metal salts. This method has been adapted for use in the assay of arylsulfatase A (ASA) and the cerebroside sulfate activator protein (
Andrew J. Norris   +16 more
doaj   +1 more source

J3-crystallin of the jellyfish lens: Similarity to saposins [PDF]

open access: yesProceedings of the National Academy of Sciences, 2001
J3-crystallin, one of the three major eye-lens proteins of the cubomedusan jellyfish ( Tripedalia cystophora ), shows similarity to vertebrate saposins, which are multifunctional proteins that bridge lysosomal hydrolases to lipids and activate enzyme activity. Sequence alignment of deduced J3-
J, Piatigorsky   +6 more
openaire   +2 more sources

Gaucher disease mouse models: point mutations at the acid β-glucosidase locus combined with low-level prosaposin expression lead to disease variants

open access: yesJournal of Lipid Research, 2005
Gaucher disease is a common lysosomal storage disease caused by a defect of acid β-glucosidase (GCase). The optimal in vitro hydrolase activity of GCase requires saposin C, an activator protein that derives from a precursor, prosaposin.
Ying Sun   +3 more
doaj   +1 more source

The Second Case of Saposin A Deficiency and Altered Autophagy [PDF]

open access: yes, 2018
Krabbe disease is a lysosomal storage disease caused by galactosylceramidase deficiency, resulting in neurodegeneration with a rapid clinical downhill course within the first months of life in the classic infantile form. This process may be triggered by the accumulation of galactosylceramide (GalCer) in nervous tissues.
Köse, Melis   +7 more
openaire   +3 more sources

Proteomic Signatures of Noise‐Induced Hearing Loss in the Mouse Cochlea

open access: yesCell Biology International, Volume 50, Issue 10, October 2026.
ABSTRACT Hearing loss affects over 1.5 billion people worldwide and has substantial social, educational, and economic consequences. Although genetic studies have identified numerous hearing‐loss‐associated genes, the molecular changes accompanying noise‐induced hearing loss (NIHL) remain incompletely understood.
Ana Carla Batissoco   +6 more
wiley   +1 more source

Structural studies of the saposins and PagP

open access: yes, 2004
This thesis comprises two parts. The first chapters describe structural studies of sphingolipid activator proteins, the saposins. This is followed by studies of the outer membrane protein PagP.
Ahn, Victoria Elizabeth
core   +2 more sources

The Lysosomal Protein Saposin B Binds Chloroquine [PDF]

open access: yesChemMedChem, 2015
AbstractChloroquine (CQ) has been widely used in the treatment of malaria since the 1950s, though toxicity and resistance is increasingly limiting its use in the clinic. More recently, CQ is also becoming recognized as an important therapeutic compound for the treatment of autoimmune disorders and has shown activity as an anticancer agent. However, the
Huta, Brian P.   +6 more
openaire   +4 more sources

A Hypertrophic Stimulus Drives Age‐Dependent Transcript Isoform Switching With and Without Differential Gene Expression in Skeletal Muscle

open access: yesAging Cell, Volume 25, Issue 10, October 2026.
A combined analysis of short‐read and long‐read RNA‐seq demonstrates reduced isoform switching after an acute hypertrophic stimulus in aged muscle and identifies age‐dependent isoforms that may not be detected with total gene‐level analyses. ABSTRACT Aged skeletal muscle has a reduced ability to adapt to hypertrophic stimuli concomitant with ...
Pieter J. Koopmans   +5 more
wiley   +1 more source

Cryo‐EM structures of multiple‐peptide resistance factor (MprF) from Pseudomonas aeruginosa

open access: yesThe FEBS Journal, Volume 293, Issue 17, Page 5318-5339, September 2026.
Multiple peptide resistance factor (MprF) is a bacterial enzyme that carries out the aminoacylation of lipid head groups. CryoEM analysis of MprF from Pseudomonas aeruginosa reveals a dimeric enzyme, distinct from its homologues in Rhizobium. The soluble GNAT domain contains a dynamic amphipathic helix (TTH) that adopts different positions in detergent
Shaileshanand Jha, Kutti R. Vinothkumar
wiley   +1 more source

Prosaposin down-modulation decreases metastatic prostate cancer cell adhesion, migration, and invasion

open access: yesMolecular Cancer, 2010
Background Factors responsible for invasive and metastatic progression of prostate cancer (PCa) remain largely unknown. Previously, we reported cloning of prosaposin (PSAP) and its genomic amplification and/or overexpression in several androgen ...
Pullikuth Ashok   +7 more
doaj   +1 more source

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