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Literature summary for 2.4.1.228 extracted from

  • Yamaji, T.; Nishikawa, K.; Hanada, K.
    Transmembrane BAX inhibitor motif containing (TMBIM) family proteins perturbs a trans-Golgi network enzyme, Gb3 synthase, and reduces Gb3 biosynthesis (2010), J. Biol. Chem., 285, 35505-35518.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

Cloned (Comment) Organism
expression analysis Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Homo sapiens 5764
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens globotriaosylceramide, Gb3, is synthesized from lactosylceramide by alpha1,4-galactosyltransferase Gb3/CD77 synthase ?
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?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
HeLa cell
-
Homo sapiens
-
JEG-3 cell choriocarcinoma cells Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information globotriaosylceramide, Gb3, is synthesized from lactosylceramide by alpha1,4-galactosyltransferase Gb3/CD77 synthase Homo sapiens ?
-
?

Synonyms

Synonyms Comment Organism
GB3 synthase
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Homo sapiens
Gb3/CD77 synthase
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Homo sapiens

General Information

General Information Comment Organism
malfunction overexpression of the truncated form of N-methyl-D-asparate-associated protein 1, GRINA-C, and some members of the full-length TMBIM family, including FAS inhibitory molecule 2, reduces Gb3, leading to accumulation of lactosylceramide. The change of glycolipid composition is restored by overexpression of Gb3 synthase, although the mRNA level of Gb3 synthase is unchanged. GRINA-C is associated with Gb3 synthase. Reduction of only the mature form of Gb3 synthase by the expression of GRINA-C is restored by addition of lysosome inhibitors, reduction of Gb3 synthase after overexpression through increased lysosomal degenaration. Phenotypes, overview Homo sapiens
physiological function globotriaosylceramide, Gb3, synthesized from lactosylceramide by alpha1,4-galactosyltransferase Gb3/CD77 synthase, is a well known receptor for Shiga toxin, Stx, produced by enterohemorrhagic Escherichia coli and Shigella dysenteriae Homo sapiens