Photoaffinity labelling of the Human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation
نویسندگان
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 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)...
متن کاملCombined replacement effects of human modified β-hexosaminidase B and GM2 activator protein on GM2 gangliosidoses fibroblasts
GM2 gangliosidoses are autosomal recessive lysosomal storage diseases (LSDs) caused by mutations in the HEXA, HEXB and GM2A genes, which encode the human lysosomal β-hexosaminidase (Hex) α- and β-subunits, and GM2 activator protein (GM2A), respectively. These diseases are associated with excessive accumulation of GM2 ganglioside (GM2) in the brains of patients with neurological symptoms. Here w...
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 کاملRabbit Anti-Ganglioside GM2 Polyclonal Antibody
BACKGROUND Introduction: GM2 is a type of ganglioside. It is associated with GM2 gangliosidoses such as Tay-Sachs disease.
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ژورنال
عنوان ژورنال: European Journal of Biochemistry
سال: 2004
ISSN: 0014-2956,1432-1033
DOI: 10.1111/j.1432-1033.2003.03964.x