A protein activator for the enzymic hydrolysis of GM2 ganglioside.
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چکیده
منابع مشابه
A protein activator for the enzymic hydrolysis of GM2 ganglioside.
A protein activator (GMz-activator) specific for stimulating the hydrolysis of G M ~ ganglioside (GalNAcb1 4GalC3 + 2aNeuAc]/?l-+ 4Glcb1-+ 1’Cer) by &hexosaminidase A has been purified over 1OS-fold with a high yield from human liver. The purification procedure includes the adjustment of the pH of liver extract to pH 4.3, followed by ammonium sulfate precipitation, Sephadex G-200 filtration, an...
متن کاملEffect of GM2 activator protein on the enzymatic hydrolysis of phospholipids and sphingomyelin.
GM2 activator protein (GM2AP) is a specific protein cofactor that stimulates the enzymatic hydrolysis of the GalNAc from GM2, a sialic acid containing glycosphingolipid, both in vitro and in lysosomes. While phospholipids together with glycosphingolipids are important membrane constituents, little is known about the possible effect of GM2AP on the hydrolysis of phospholipids. Several recent rep...
متن کاملGanglioside GM2-activator protein and vesicular transport in collecting duct intercalated cells.
This study describes the molecular characterization of an antigen defined by an autoantibody from a woman with habitual abortion as GM2-activator protein. The patient showed no disorder of renal function. Accidentally with routine serum screening for autoantibodies, an immunoreactivity was found in kidney collecting duct intercalated cells. Three distinct patterns of immunostaining of intercala...
متن کاملIn Cellulo Examination of a Beta-Alpha Hybrid Construct of Beta-Hexosaminidase A Subunits, Reported to Interact with the GM2 Activator Protein and Hydrolyze GM2 Ganglioside
The hydrolysis in lysosomes of GM2 ganglioside to GM3 ganglioside requires the correct synthesis, intracellular assembly and transport of three separate gene products; i.e., the alpha and beta subunits of heterodimeric beta-hexosaminidase A, E.C. # 3.2.1.52 (encoded by the HEXA and HEXB genes, respectively), and the GM2-activator protein (GM2AP, encoded by the GM2A gene). Mutations in any one o...
متن کاملTwo polymorphisms for the human GM2 activator protein gene.
Source/Description: A 300 bp HincII/EcoRI fragment of the human G^ activation protein cDNA (1) was subcloned into Bluescript vector and used as a probe. Polymorphism: Mspl identifies a two allele polymorphism with bands at either 3.4 kb (Al) or 2.9 kb (A2) and invariant bands at 1.0 kb, 1.8 kb and 4.0 kb. Rsal identifies a two allele polymorphism with a band at either 4.1 kb (Bl) or 3.4 kb (B2)...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1981
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(19)69152-0