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Isolation of a cDNA encoding the human GM2 activator protein.
Institut für Organische Chemie und Biochemie, Bonn, FRG.
The GM2 activator protein is a glycolipid-binding protein required for the lysosomal degradation of ganglioside GM2. A human fibroblast cDNA library was screened with mixtures of oligonucleotide probes corresponding to four different areas of the amino acid sequence. A putative clone (821 bp) which gave positive signals to all four probe mixtures was purified and sequenced. The sequence was colinear with the sequence of 160 amino acids of the mature GM2 activator protein. Availability of the cDNA clone should facilitate investigation into function of the GM2 activator protein and also into genetic abnormalities underlying GM2 gangliosidosis AB variant.
PMID: 2753159 [PubMed - indexed for MEDLINE]
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Cited by 7 PubMed Central articles
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Effect of structural modifications of ganglioside GM2 on intra-molecular carbohydrate-to-carbohydrate interaction and enzymatic susceptibility.
Li YT, Li SC, Kiso M, Ishida H, Mauri L, Raimondi L, Bernardi A, Sonnino S.
Biochim Biophys Acta. 2008 Mar; 1780(3):353-61. Epub 2007 Oct 5.
[Biochim Biophys Acta. 2008]
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Identification of a lysosomal protein causing lipid transfer, using a fluorescence assay designed to monitor membrane fusion between rat liver endosomes and lysosomes.
Kuwana T, Mullock BM, Luzio JP.
Biochem J. 1995 Jun 15; 308 ( Pt 3):937-46.
[Biochem J. 1995]
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Over-expression of a functionally active human GM2-activator protein in Escherichia coli.
Klima H, Klein A, van Echten G, Schwarzmann G, Suzuki K, Sandhoff K.
Biochem J. 1993 Jun 1; 292 ( Pt 2):571-6.
[Biochem J. 1993]
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