Biosynthesis and deficiencies of glycosylphosphatidylinositol

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Biosynthesis and deficiencies of glycosylphosphatidylinositol

At least 150 different human proteins are anchored to the outer leaflet of the plasma membrane via glycosylphosphatidylinositol (GPI). GPI preassembled in the endoplasmic reticulum is attached to the protein's carboxyl-terminus as a post-translational modification by GPI transamidase. Twenty-two PIG (for Phosphatidyl Inositol Glycan) genes are involved in the biosynthesis and protein-attachment...

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Biosynthesis of glycosylphosphatidylinositol membrane anchors.

Covalent modification of proteins with phosphatidylinositol (PI) was first suggested as a novel mechanism of membrane attachment when it was found that PI-specific phospholipase C (PI-PLC) could release alkaline phosphatase from tissue slices [1-5]. Subsequent compositional analysis of Trypanosoma brucei variant surface glycoprotein (VSG) [6,7], Torpedo acetylcholinesterase [8], erythrocyte ace...

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Mutations in PIGY: expanding the phenotype of inherited glycosylphosphatidylinositol deficiencies

Glycosylphosphatidylinositol (GPI)-anchored proteins are ubiquitously expressed in the human body and are important for various functions at the cell surface. Mutations in many GPI biosynthesis genes have been described to date in patients with multi-system disease and together these constitute a subtype of congenital disorders of glycosylation. We used whole exome sequencing in two families to...

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Genome-Wide Screening of Genes Required for Glycosylphosphatidylinositol Biosynthesis

Glycosylphosphatidylinositol (GPI) is synthesized and transferred to proteins in the endoplasmic reticulum (ER). GPI-anchored proteins are then transported from the ER to the plasma membrane through the Golgi apparatus. To date, at least 17 steps have been identified to be required for the GPI biosynthetic pathway. Here, we aimed to establish a comprehensive screening method to identify genes i...

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Inhibition of glycosylphosphatidylinositol biosynthesis in Leishmania mexicana by mannosamine.

In several Leishmania species, glycosylated inositol phospholipids exist as free lipids, as membrane protein anchors, and as the membrane-binding moieties of the lipophosphoglycans. Both the glycolipid-anchored cell surface metalloproteinase, gp63, and the lipophosphoglycans have been proposed to be involved in cell invasion. Moreover, the lipophosphoglycans have been implicated in the survival...

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ژورنال

عنوان ژورنال: Proceedings of the Japan Academy, Series B

سال: 2014

ISSN: 0386-2208,1349-2896

DOI: 10.2183/pjab.90.130