serw-MX  [xml]  

 DeCS Categories

C10 Nervous System Diseases .
C10.228 Central Nervous System Diseases .
C10.228.140 Brain Diseases .
C10.228.140.163 Brain Diseases, Metabolic .
C10. Brain Diseases, Metabolic, Inborn .
C10. Lysosomal Storage Diseases, Nervous System .
C10. Sphingolipidoses .
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities .
C16.320 Genetic Diseases, Inborn .
C16.320.565 Metabolism, Inborn Errors .
C16.320.565.189 Brain Diseases, Metabolic, Inborn .
C16.320.565.189.435 Lysosomal Storage Diseases, Nervous System .
C16.320.565.189.435.825 Sphingolipidoses .
C16.320.565.398 Lipid Metabolism, Inborn Errors .
C16.320.565.398.641 Lipidoses .
C16.320.565.398.641.803 Sphingolipidoses .
C16.320.565.595 Lysosomal Storage Diseases .
C16.320.565.595.554 Lysosomal Storage Diseases, Nervous System .
C16.320.565.595.554.825 Sphingolipidoses .
C18 Nutritional and Metabolic Diseases .
C18.452 Metabolic Diseases .
C18.452.132 Brain Diseases, Metabolic .
C18.452.132.100 Brain Diseases, Metabolic, Inborn .
C18.452.132.100.435 Lysosomal Storage Diseases, Nervous System .
C18.452.132.100.435.825 Sphingolipidoses .
C18.452.584 Lipid Metabolism Disorders .
C18.452.584.687 Lipidoses .
C18.452.584.687.803 Sphingolipidoses .
C18.452.648 Metabolism, Inborn Errors .
C18.452.648.189 Brain Diseases, Metabolic, Inborn .
C18.452.648.189.435 Lysosomal Storage Diseases, Nervous System .
C18.452.648.189.435.825 Sphingolipidoses .
C18.452.648.398 Lipid Metabolism, Inborn Errors .
C18.452.648.398.641 Lipidoses .
C18.452.648.398.641.803 Sphingolipidoses .
C18.452.648.595 Lysosomal Storage Diseases .
C18.452.648.595.554 Lysosomal Storage Diseases, Nervous System .
C18.452.648.595.554.825 Sphingolipidoses .
D08 Enzymes and Coenzymes .
D08.211 Coenzymes .
D08.211.790 Sphingolipid Activator Proteins .
D08.211.790.500 Saposins .
D10 Lipids .
D10.570 Membrane Lipids .
D10.570.877 Sphingolipids .
 Synonyms & Historicals
Sphingolipid Activator Proteins .
Cerebroside Sulfate Activator Protein .
G(M1) Ganglioside Activating Factor .
GM1 Activator Protein .
Ganglioside Degradation Activator Protein .
Ganglioside Hydrolysis Activator Proteins .
Hexosaminidase A Activating Protein .
Hexosaminidase Activating Factor .
G(M1) Activator Protein .
A family of glycoprotein cofactors that are required for the efficient catabolization of SPHINGOLIPIDS by specific acid hydrolases such as GLUCOSYLCERAMIDASE; GALACTOCEREBROSIDASE; BETA-N-ACETYLHEXOSAMINIDASE; and CEREBROSIDE-SULFATASE. .
Saposins .
Co-beta-Glucosidase .
Coglucosidase .
Gaucher Activator Protein .
Glucosylceramidase Activator .
SAP-1 Sphingolipid Activator .
SAP-A Protein .
SAP-C Protein .
SAP-D Protein .
Saposin A .
Saposin B .
Saposin C .
Saposin D .
Sphingolipid Activator Protein 1 .
Sphingolipid Activator Protein 2 .
Sphingolipid Activator Protein-1 .
Testibumin .
beta-Glucosidase Activator Protein .
beta-Glucosidase Stimulating Protein .
Co beta Glucosidase .
SAP 1 Sphingolipid Activator .
SAP A Protein .
SAP C Protein .
SAP D Protein .
Sphingolipid Activator, SAP-1 .
beta Glucosidase Activator Protein .
beta Glucosidase Stimulating Protein .
A group of four homologous sphingolipid activator proteins that are formed from proteolytic cleavage of a common protein precursor molecule referred to as prosaposin. .
Sphingolipids .
Lysosphingolipids .
A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed) .
Sphingolipidoses .
Sphingolipid Storage Diseases .
Sphingolipidosis .
Sphingolipid Storage Disease .
Storage Disease, Sphingolipid .
Storage Diseases, Sphingolipid .
A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign. .