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PMID: 8861954 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

PIG-B, a membrane protein of the endoplasmic reticulum with a large lumenal domain, is involved in transferring the third mannose of the GPI anchor.

The EMBO journal ·Vol. 15 ·No. 16 ·1996-08-15 ·Pages 4254-61

Takahashi M, Inoue N, Ohishi K, Maeda Y, Nakamura N, Endo Y, Fujita T, Takeda J, Kinoshita T

Abstract

Many eukaryotic cell surface proteins are bound to the membrane via the glycosylphosphatidylinositol (GPI) anchor that is covalently linked to their carboxy-terminus. The GPI anchor precursor is synthesized in the endoplasmic reticulum (ER) and post-translationally linked to protein. We cloned a human gene termed PIG-B (phosphatidylinositol glycan of complementation class B) that is involved in transferring the third mannose. PIG-B encodes a 554 amino acid, ER transmembrane protein with an amino-terminal portion of approximately 60 amino acids on the cytoplasmic side and a large carboxy-terminal portion of 470 amino acids within the ER lumen. A mutant PIG-B lacking the cytoplasmic portion remains active, indicating that the functional site of PIG-B resides on the lumenal side of the ER membrane. The PIG-B gene was localized to chromosome 15 at q21-q22. This autosomal location would explain why PIG-B is not involved in the defective GPI anchor synthesis in paroxysmal nocturnal hemoglobinuria, which is always caused by a somatic mutation of the X-linked PIG-A gene.

MeSH Terms
Amino Acid Sequence Animals Base Sequence Chromosome Mapping Chromosomes, Human, Pair 15/genetics Cloning, Molecular DNA, Complementary/genetics Dolichol Monophosphate Mannose/metabolism Endoplasmic Reticulum/metabolism Genes Glycosylphosphatidylinositols/deficiency,metabolism Humans Mannose/metabolism Mannosyltransferases/genetics,metabolism,physiology Mice Molecular Sequence Data Recombinant Fusion Proteins/metabolism Sequence Deletion Thymoma/genetics,pathology Thymus Neoplasms/genetics,pathology
Chemicals
DNA, Complementary Glycosylphosphatidylinositols Recombinant Fusion Proteins Dolichol Monophosphate Mannose Mannosyltransferases PIGB protein, human dolichyl-phosphate beta-D-mannosyltransferase Mannose
Authors & Affiliations
9 authors, click to expand affiliations / ORCID
Takahashi M
Department of Immunoregulation, Research Institute for Microbial Diseases, Osaka University, Suita, Japan.
Inoue N
Ohishi K
Maeda Y
Nakamura N
Endo Y
Fujita T
Takeda J
Kinoshita T
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Article Info
Journal
The EMBO journal
Abbr.
EMBO J
ISSN
0261-4189
Published
1996-08-15
Pages
4254-61
Language
English
Region
England
NLM ID
8208664
PMCID
PMC452151
Subset
IM
Databases
GENBANK
D42138, D84436
External Links
PubMed source PMC Full Text View journal details
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