Complete localization of disulfide bonds in GM2 activator protein

Protein Science

Volumn 7, Issue 4, 1998, Pages 1039-1045

  Schütte, Christina G ^a   Lemm, Thorsten ^a   Glombitza, Gereon J ^b   Sandhoff, Konrad ^a  

^a UNIVERSITY OF BONN   (Germany)

^b MAX PLANCK INSTITUTE FOR CHEMICAL ECOLOGY   (Germany)

Author keywords

Disulfide bonds; GM2 activator protein; GM2 gangliosidosis AB variant, MADLI MS; PSD

Indexed keywords

BETA N ACETYLHEXOSAMINIDASE A; CYSTEINE; GANGLIOSIDE GM2; SPHINGOLIPID ACTIVATOR PROTEIN;

ARTICLE; CELL DEATH; CHROMOSOME 10; CHROMOSOME 5; DISULFIDE BOND; ENZYME DEGRADATION; ESCHERICHIA COLI; GM2 GANGLIOSIDOSIS; LYSOSOME; MASS SPECTROMETRY; NONHUMAN; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN DOMAIN; PROTEIN FOLDING; PROTEIN LOCALIZATION; STAPHYLOCOCCUS AUREUS; TAY SACHS DISEASE;

ESCHERICHIA COLI; STAPHYLOCOCCUS AUREUS;

EID: 0039703311     PISSN: 09618368     EISSN: None     Source Type: Journal    
DOI: 10.1002/pro.5560070421     Document Type: Article

References (25)

1. Burg J, Conzelmann E, Sandhoff K. 1985. Mapping the gene coding for the human GM2 activator protein to chromosome 5. Ann Hum Genet 49:41-45.
2. Conzelmann E, Sandhoff K. 1978. AB variant of infantile GM2 gangliosidosis: Deficiency of a factor necessary for stimulation of hexosammidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2. Proc Natl Acad Sci USA 8:3979-3983.
3. Fürst W, Machleidt W, Sandhoff K. 1988. The precursor of sulfatide activator protein is processed to three different proteins. Biol Chem Hoppe-Seyler 369:317-328.
4. Fürst W, Schubert J, Machleidt W, Meyer HE, Sandhoff K. 1990. The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein. Eur J Biochem 192:709-713.
5. Glombitza GJ, Becker E, Kaiser HW, Sandhoff K. 1997. Biosynthesis, processing and intracellular transport of GM2 activator protein in human epidermal keratinocytes: The lysosomal targeting of GM2 activator is independent of a mannose-6-phosphate signal. J Biol Chem 272:5199-5208.
6. Gorman JJ, Ferguson BL, Speelman D, Mills J. 1997. Determination of the disulfide bond arrangement of human respiratory syncytial virus attachment (G) protein by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. Protein Sci 6:1308-1315.
7. Hochuli E, Bannwarth W, Döbeli H, Gentz R, Stuber D. 1988. Genetic approach to facilitate purification of recombinant proteins with a novel metal chelate absorbent. Biotechnology 6:1321-1325.
8. Kaufmann R, Kirsch D, Spengler B. 1994. Sequencing of peptides in a time-of-flight mass spectrometer: Evaluation of postsource decay following matrix-assisted laser desorption ionization (MALDI). Int J Mass Spectrom Ion Proc 86:137-154.
9. Klima H, Klein A, van Echten G, Schwarzmann G, Suzuki K, Sandhoff K. 1993. Over-expression of a functionally active human GM2 activator protein in Escherichia coli. Biochem J 292:571-576.
10. Klima H, Tanaka A, Schnabel D, Nakano T, Schröder M, Suzuki K, Sandhoff K. 1991. Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein. FEBS Lett 289:260-264.
11. Nagarajan S, Chen H-C-, Li S-C, Li Y-T, Lockyer JM. 1992. Evidence for two cDNA clones encoding human GM2-activator protein. Biochem J 282:807-813.
12. Nakano T, Sandhoff K, Stümper J, Christomanou H, Suzuki K. 1989. Structure and full-length cDNA coding for sulfatide activator, a co-β-glucosidase and two other homologous proteins: Two alternate forms of the sulfatide activator. J Biochem 105:152-154.
13. O'Brien JS, Kretz KA, Dewji NN, Wenger DA, Esch F, Fluharty AL. 1988. Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus Science 241:1098-1101.
14. Patterson SD, Katta V. 1994. Prompt fragmentation of disulfide-linked peptides during matrix-assisted laser desorption ionization mass spectrometry. Anal Chem 66:3737-3732.
15. Sandhoff K, Harzer K, Fürst W. 1992. Sphingolipid activator proteins. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic bases of inherited disease New York: McGraw Hill. pp 2427-2443.
16. Schröder M, Schnabel D, Suzuki K, Sandhoff K. 1991. A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB. FEBS Lett 290:1-3.
17. Smiljanic-Georgijev N, Rigat B, Xie B, Wang W, Mahuran DJ. 1997. Characterization of the affinity of the GM2 activator protein for glycolipids by fluorescence dequenching assay. Biochim Biophys Acta 1339:192-202.
18. Stultz CM, White JV, Smith TF. 1993. Structural analysis based on state-space modeling. Protein Sci 2:305-314.
19. Vacaro AM, Salvioli R, Barca A, Tatti M, Ciaffno F, Maras B, Sciliano, R, Zappacosta F, Amoresano A, Pucci P. 1995. Structural analysis of saposin C and B: Complete localization of disulfide bridges. J Biol Chem 270:9953-9960.
20. White JV, Stultz CM, Smith TF. 1994. Protein classification by stochastic modeling and optimal filtering of amino-acid sequences. Math Biosci 119:35-75.
21. Wu F, Watson JT. 1997. A novel methodology for assignment of disulfide bond pairings in proteins. Protein Sci 6:391-398.
22. Wu Y-Y, Sonnino S, Li Y-T, Li S-C. 1996a. Expression and specificity of human GM2 activator protein. J Biol Chem 169:16276-16283.
23. Wu Y-Y, Sonnino S, Li Y-T, Li S-C. 1996b. Characterization of an alternatively spliced GM2 activator protein, GM2A protein. J Biol Chem 271:10611-10615.
24. Xie B, McInnes B, Neote K, Lamhonwah A-M, Mahuran DJ. 1991. Isolation and expression of a full-length cDNA encoding the human GM2 activator protein. Biochem Biophys Res Commun 177:1217-1223.
25. Xie B, Wang W, Mahuran D. 1992. A Cys138-to Arg substitution in the GM2 activator protein is associated with the AB variant form of GM2 gangliosidosis. Ann J Hum Genet 50:1046-1052.