Results 1 to 10 of about 10,573 (148)
Sphingolipid Activator Proteins Are Required for Epidermal Permeability Barrier Formation [PDF]
The epidermal permeability barrier is maintained by extracellular lipid membranes within the interstices of the stratum corneum. Ceramides, the major components of these multilayered membranes, derive in large part from hydrolysis of glucosylceramides mediated by stratum corneum beta-glucocerebrosidase (beta-GlcCerase).
Walter M Holleran +2 more
exaly +5 more sources
Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease
The lysosomal degradation of glucosylceramide requires the hydrolase, glucosylceramide‐β‐glucosidase and a sphingolipid activator protein (Gaucher factor, SAP‐2, saposin C). Genetic defects in either of these lysosomal proteins cause phenotypically similar disorders in man, the Gaucher disease.
Konrad Sandhoff, Martin Schröder
exaly +4 more sources
Dissecting the regulatory roles of ORM proteins in the sphingolipid pathway of plants.
Sphingolipids are a vital component of plant cellular endomembranes and carry out multiple functional and regulatory roles. Different sphingolipid species confer rigidity to the membrane structure, facilitate trafficking of secretory proteins, and ...
Adil Alsiyabi +3 more
doaj +3 more sources
Sphingolipid activator proteins (saposins A, B, C, and D) are derived from a common precursor protein (prosaposin) and specifically activate in vivo degradation of glycolipids with short carbohydrate chains.
Keiko Tadano-Aritomi +4 more
doaj +3 more sources
Identification of the binding and activating sites of the sphingolipid activator protein, saposin C, with glucocerebrosidase [PDF]
AbstractSaposin C is a sphingolipid activator protein of 8.5 kDa that activates lysosomal glucocerebrosidase. Previously, we synthesized and characterized a synthetic full‐length human saposin C protein that displays 85% of the activity of the native saposin C.
John M Tomich +2 more
exaly +3 more sources
Lysosomal Storage Diseases: Heterogeneous Group of Disorders [PDF]
The name of lysosomal storage diseases stems from the fact that in this category of disorders specific undegraded materials are stored in the lysosomes.
David A. Wenger +2 more
doaj +1 more source
Aflatoxins are immunosuppressive and carcinogenic secondary metabolites, produced by the filamentous ascomycete Aspergillus flavus, that are hazardous to animal and human health.
Kalyani Prasad +6 more
doaj +1 more source
A prosaposin-derived Peptide alleviates kainic Acid-induced brain injury. [PDF]
Four sphingolipid activator proteins (i.e., saposins A-D) are synthesized from a single precursor protein, prosaposin (PS), which exerts exogenous neurotrophic effects in vivo and in vitro.
Hiroaki Nabeka +7 more
doaj +1 more source
A Förster resonance energy transfer-based fusion and transfer assay was developed to study, in model membranes, protein-mediated membrane fusion and intermembrane lipid transfer of fluorescent sphingolipid analogs.
Günter Schwarzmann +2 more
doaj +1 more source

