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Sphingolipidoses (Sphingolipidosis)

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.
Also Known As:
Sphingolipidosis; Sphingolipid Storage Diseases; Sphingolipid Storage Disease; Storage Disease, Sphingolipid; Storage Diseases, Sphingolipid
Networked: 134 relevant articles (1 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Glycogen Storage Disease Type II (Pompe's Disease)
2. Mucopolysaccharidoses
3. Mucolipidoses (Sialidosis)
4. Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis)
5. Pathologic Processes

Experts

1. Futerman, Anthony H: 6 articles (06/2015 - 08/2002)
2. Sandhoff, Konrad: 5 articles (05/2014 - 07/2007)
3. Suzuki, Yoshiyuki: 5 articles (04/2013 - 08/2004)
4. Ohno, Kousaku: 5 articles (04/2013 - 08/2004)
5. Ninomiya, Haruaki: 4 articles (11/2010 - 08/2004)
6. Ogawa, Seiichiro: 4 articles (11/2010 - 08/2004)
7. Pagano, Richard E: 4 articles (01/2005 - 11/2002)
8. Luan, Zhuo: 3 articles (04/2013 - 01/2010)
9. Ikonen, Elina: 3 articles (09/2012 - 02/2003)
10. Iida, Masami: 3 articles (11/2010 - 05/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sphingolipidoses:
1. CholesterolIBA
2. SphingolipidsIBA
3. LipidsIBA
4. EnzymesIBA
5. HydrolasesIBA
6. GlycosphingolipidsIBA
7. Proteins (Proteins, Gene)IBA
8. CeroidIBA
9. methylamine (methylammonium)IBA
10. rab GTP-Binding ProteinsIBA

Therapies and Procedures

1. Enzyme Replacement Therapy
2. Cell Transplantation
3. Lasers (Laser)
4. Kidney Transplantation
5. Transplantation (Transplant Recipients)