serw-MX  [xml]  
 


    
 DeCS Categories

A11 Cells .
A11.284 Cellular Structures .
A11.284.149 Cell Membrane .
A11.284.149.165 Cell Membrane Structures .
A11.284.149.165.570 Membrane Microdomains .
C10 Nervous System Diseases .
C10.228 Central Nervous System Diseases .
C10.228.140 Brain Diseases .
C10.228.140.163 Brain Diseases, Metabolic .
C10.228.140.163.100 Brain Diseases, Metabolic, Inborn .
C10.228.140.163.100.435 Lysosomal Storage Diseases, Nervous System .
C10.228.140.163.100.435.825 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 .
D09 Carbohydrates .
D09.400 Glycoconjugates .
D09.400.410 Glycolipids .
D09.400.410.420 Glycosphingolipids .
D10 Lipids .
D10.390 Glycolipids .
D10.390.470 Glycosphingolipids .
D10.570 Membrane Lipids .
D10.570.877 Sphingolipids .
D10.570.877.360 Glycosphingolipids .
 
 Terms
 Synonyms & Historicals
Documents
LILACS e MDL
 
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) .
1.00
93538
 
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. .
0.86
4346
 
Glycosphingolipids .
Asialogangliosides .
Sphingoglycolipids .
Lipids containing at least one monosaccharide residue and either a sphingoid or a ceramide (CERAMIDES). They are subdivided into NEUTRAL GLYCOSPHINGOLIPIDS comprising monoglycosyl- and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides; and ACIDIC GLYCOSPHINGOLIPIDS which comprises sialosylglycosylsphingolipids (GANGLIOSIDES); SULFOGLYCOSPHINGOLIPIDS (formerly known as sulfatides), glycuronoglycosphingolipids, and phospho- and phosphonoglycosphingolipids. (From IUPAC's webpage) .
0.77
213941
 
Membrane Microdomains .
Sphingolipid Microdomains .
Sphingolipid-Cholesterol Rafts .
Membrane Microdomain .
Microdomain, Membrane .
Microdomain, Sphingolipid .
Microdomains, Membrane .
Microdomains, Sphingolipid .
Sphingolipid Cholesterol Rafts .
Sphingolipid Microdomain .
Sphingolipid-Cholesterol Raft .
Lipid Rafts, Cell Membrane .
Detergent-insoluble CELL MEMBRANE components. They are enriched in SPHINGOLIPIDS and CHOLESTEROL and clustered with glycosyl-phosphatidylinositol (GPI)-anchored proteins. .
0.70
67046
 
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. .
0.68
0207