Sulfatidosis

A group of inherited metabolic disorders characterized by the intralysosomal accumulation of sulfur-containing lipids (SULFATIDES), including SULFOGLYCOSPHINGOLIPIDS normally found in the MYELIN SHEATH of the brain. These disorders are caused by defective degradative enzymes leading to substrate accumulation (or storage).

Also Known As:

Networked: 3 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Metachromatic Leukodystrophy (Sulfatide Lipidosis)

Experts

1.	Asfaw, Befekadu: 1 article (10/2013)
2.	Honzíková, Jitka: 1 article (10/2013)
3.	Kuchař, Ladislav: 1 article (10/2013)
4.	Ledvinová, Jana: 1 article (10/2013)
5.	Poupětová, Helena: 1 article (10/2013)
6.	Tureček, František: 1 article (10/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sulfatidosis:

1.	Sulfoglycosphingolipids (Sulfatides)IBA 10/21/2013 - "Urinary sulfatides were analyzed in a set of 21 samples from patients affected by sulfatidosis. " 07/01/1971 - "Human urinary sulfatides in patients with sulfatidosis (metachromatic leukodystrophy)." 06/01/1974 - "Deficiency of lactosyl sulfatide sulfatase in metachromatic leucodystrophy (sulfatidosis)." 07/01/1971 - "The determination of urinary sulfatides was a valuable confirmation of the deficiency in arylsulfatase A activity characteristic of sulfatidosis." 07/01/1971 - "On comparing samples from normal individuals and patients with sulfatidosis, urinary sulfatide composition was remarkably similar to that previously reported in the kidney, including differences in fatty acid pattern. "
2.	SulfatasesIBA 06/01/1974 - "Deficiency of lactosyl sulfatide sulfatase in metachromatic leucodystrophy (sulfatidosis)."
3.	LipidsIBA 07/01/1971 - "In sulfatidosis, a hereditary sulfatide storage disorder known as metachromatic leukcdystrophy, a large increase in sulfatide was readily apparent on a thin-layer chromatogram of the crude lipid extract. "
4.	Fatty Acids (Saturated Fatty Acids)IBA 07/01/1971 - "On comparing samples from normal individuals and patients with sulfatidosis, urinary sulfatide composition was remarkably similar to that previously reported in the kidney, including differences in fatty acid pattern. "
5.	Cerebroside-Sulfatase (Arylsulfatase A)IBA 07/01/1971 - "The determination of urinary sulfatides was a valuable confirmation of the deficiency in arylsulfatase A activity characteristic of sulfatidosis."