Sphingolipid Activator Proteins Are Required for Epidermal Permeability Barrier Formation [PDF]
Journal of Biological Chemistry, 1999 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).
Peter Elias +2 more
exaly +6 more sources
Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease [PDF]
FEBS Letters, 1991 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
exaly +4 more sources
Dissecting the regulatory roles of ORM proteins in the sphingolipid pathway of plants. [PDF]
PLoS Computational Biology, 2021 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
Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice [PDF]
Journal of Lipid Research, 2003 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
[29] Sphingolipid hydrolases and activator proteins [PDF]
Biochimie, 2000 The physiological degradation of several membrane-bound glycosphingolipids (GSLs) by water-soluble lysosomal exohydrolases requires the assistance of sphingolipid activator proteins (SAPs). Four of these SAPs are synthesized from a single precursor protein (prosaposin).
Uwe Bierfreund +2 more
core +7 more sources
Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases [PDF]
Disease Models & Mechanisms, 2017 Sphingolipidoses are inherited diseases belonging to the class of lysosomal storage diseases (LSDs), which are characterized by the accumulation of indigestible material in the lysosome caused by specific defects in the lysosomal degradation machinery ...
Julia Sellin +10 more
doaj +2 more sources
Identification of the binding and activating sites of the sphingolipid activator protein, saposin C, with glucocerebrosidase [PDF]
Protein Science, 1995 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.
, J A Barranger, J S O'Brien
exaly +3 more sources
The lysosomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin [PDF]
The EMBO Journal, 2003 Most soluble lysosomal proteins bind the mannose 6-phosphate receptor (M6P-R) to be sorted to the lysosomes. However, the lysosomes of I-cell disease (ICD) patients, a condition resulting from a mutation in the phosphotransferase that adds mannose 6-phosphate to hydrolases, have near normal levels of several lysosomal proteins, including the ...
Stephane, Lefrancois +4 more
openaire +4 more sources
Sphingolipid activator proteins: proteins with complex functions in lipid degradation and skin biogenesis [PDF]
Glycobiology, 2001 Sphingolipid activator proteins (SAPs or saposins) are essential cofactors for the lysosomal degradation of membrane-anchored sphingolipids. Four of the five known proteins of this class, SAPs A--D, derive from a single precursor protein and show high homology, whereas the fifth protein, GM2AP, is larger and displays a different secondary structure ...
Schuette, C. G. +3 more
openaire +5 more sources
Lysosomal Storage Diseases: Heterogeneous Group of Disorders [PDF]
BioImpacts, 2013 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