Results 1 to 10 of about 1,665,622 (222)
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 +2 more sources
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).
T, Doering +6 more
openaire +4 more sources
The lysosomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin [PDF]
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 ...
Stéphane Lefrançois, Morales CARLOS R
exaly +3 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.
John A Barranger, J S O'Brien
exaly +3 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
Toxins, 2023 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
Functional characterisation of the lipid raft protein stomatin [PDF]
, 2005 The function of the integral membrane protein stomatin is as yet poorly understood. Stomatin is deficient from the erythrocyte membrane of patients suffering with Overhydrated Hereditary Stomatocytosis (OHSt).
Wilkinson, Dorothy Kate
core +7 more sources
A prosaposin-derived Peptide alleviates kainic Acid-induced brain injury. [PDF]
PLoS ONE, 2015 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