Biomaterial Database

Enzymatic study of GM-1 gangliosidosis. 1975

T Yutaka, and S Okada, and K Mimaki, and T Sugita, and H Yabuuchi

Two types of GM-1 gangliosidosis were studied biochemically. Type 1 liver accumulated non-lipid hexosamine in addition to GM-1 ganglioside, but there was no increase of hexosamine and GM-1 in type 2 liver. The optimum pH of liver beta-galactosidase of type 1 and type 2 was 5--6 while that of the normal control was 4.5. Type 1 brain beta-galactosidase showed a slightly acidic optimum pH, i.e. 4.0 in comparison with that of the normal control. The optimum pH of type 2 brain beta-galactosidase was 5.5, like the liver enzyme. The thermostability of liver beta-galactosidase was the same in type 1 and type 2, while that of brain was different. Beta-galactosidase of type 1 and type 2 liver is more stable at 42 degrees C than the normal control, but a different thermostability was observed in type 1 and type 2 brain. Liver beta-galactosidase of type 1 showed one peak each at acid and neutral pH, and type 2 liver had only one peak at neutral pH.

UI	MeSH Term	Description	Entries
D008052	Lipid Metabolism, Inborn Errors	Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.	Lipid Metabolism, Inborn Error
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008297	Male		Males
D009438	Neuraminic Acids	Nine-carbon deoxyamino SUGAR ACIDS that are substrates for NEURAMINIDASE. Their derivatives include SIALIC ACIDS.	Acids, Neuraminic
D009928	Organ Specificity	Characteristic restricted to a particular organ of the body, such as a cell type, metabolic response or expression of a particular protein or antigen.	Tissue Specificity,Organ Specificities,Specificities, Organ,Specificities, Tissue,Specificity, Organ,Specificity, Tissue,Tissue Specificities
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D005696	Galactosidases	A family of galactoside hydrolases that hydrolyze compounds with an O-galactosyl linkage. EC 3.2.1.-.	Galactosidase
D005732	Gangliosides	A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)	Ganglioside,Sialoglycosphingolipids