Results 111 to 120 of about 1,761,902 (161)

Sphingolipid metabolism-related genes B4GALNT1 and CERS4 as prognostic biomarkers in lung adenocarcinoma. [PDF]

PLoS One
Jeon J, Kang J, Park JH, Lee JH, Kim DE, Park WJ, Kim S.   +6 more
europepmc   +1 more source

Activator proteins and topology of lysosomal sphingolipid catabolism

Lipids and Lipid Metabolism, 1992
The lysosomal degradation of several sphingolipids by acid hydrolases is dependent on small non-enzymic cofactors, called sphingolipid activator proteins some of which have been identified as sphingolipid binding proteins. This review summarizes the information available on the structure, function, biosynthesis, gene organization and pathobiochemistry ...
Konrad Sandhoff, W Fürst, Werner Fürst
exaly   +3 more sources

Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins

Chemistry and Physics of Lipids, 1999
Lysosomal breakdown of glycosphingolipids with short hydrophilic carbohydrate headgroups is achieved by the simultaneous action of specific hydrolases and sphingolipid activator proteins (SAPs). Activator proteins are considered to facilitate the enzyme/substrate interaction between water-soluble enzymes and membrane-bound substrates.
Konrad Sandhoff, Thomas Linke, Klaus Ferlinz   +2 more
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 S O'Brien, John A Barranger
exaly   +3 more sources

Sphingolipid hydrolase activator proteins and their precursors

Biochemical and Biophysical Research Communications, 1989
Activator proteins for sphingolipid hydrolases (saposins) are small acidic, heat-stable glycoproteins that stimulate the hydrolysis of sphingolipids by lysosomal enzymes. The molecular mass of each stimulator is about 10 kDa, but glycosylated forms of higher mass exist too.
A, Sano, T, Hineno, T, Mizuno, K, Kondoh, S, Ueno, Y, Kakimoto, K, Inui   +6 more
openaire   +2 more sources

Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

Biochimica Et Biophysica Acta - Molecular Basis of Disease, 2000
We have investigated the mRNA amounts of six lysosomal proteins (β-hexosaminidase α- and β-subunit, sphingolipid activator protein precursor, GM2 activator protein, lysosomal sialidase, β-glucocerebrosidase) involved in the degradation of ...
Richard L. Proia, Konrad Sandhoff, Kunihiko Suzuki   +2 more
exaly   +2 more sources

Saposins (Sphingolipid Activator Proteins) in the Twitcher Mutant Mouse

Journal of Neurochemistry, 1990
Abstract: The twitcher mutant mouse, the animal model of Krabbe disease (human globoid cell leukodystrophy), is characterized by apparent deficiency of galactosylceramide β‐galactosidase activity. Saposin A and C, the heat‐stable small sphingolipid activator glycoproteins, stimulate the activity of galactosylceramide β‐galactosidase as well as ...
Andrew M Yeager, John S O'Brien
exaly   +3 more sources

Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease

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   +3 more sources

PRINCIPLES OF LYSOSOMAL MEMBRANE DIGESTION: Stimulation of Sphingolipid Degradation by Sphingolipid Activator Proteins and Anionic Lysosomal Lipids

Annual Review of Cell and Developmental Biology, 2005
Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and intra-lysosomal membrane structures. During endocytosis, these intra-lysosomal membranes are formed and prepared for digestion by a lipid-sorting process during which their ...
Thomas Kolter, Konrad Sandhoff
exaly   +3 more sources