NDLI: Goodpasture Antigen-binding Protein, the Kinase That Phosphorylates the Goodpasture Antigen, Is an Alternatively Spliced Variant Implicated in Autoimmune Pathogenesis

Content Provider	Journal of Biological Chemistry (JBC)
Author	Raya, Angel Revert-Ros, Francisco Martinez-Martinez, Pilar Navarro, Samuel Roselló, Esther Vieites, Begoña Granero, Froilán Forteza, Jerónimo Saus, Juan
Description	Goodpasture antigen-binding protein and its alternative splicing variant devoid of the exon-encoded 26-residue motif (GPBP and GPBPΔ26)
Abstract	The non-collagenous C-terminal domain of the α3 chain of collagen IV is the autoantigen in Goodpasture disease, an autoimmune disorder described only in humans. Specific N-terminal phosphorylation is a biological feature unique to the human domain when compared with other homologous domains lacking immunopathogenic potential. We have recently cloned from a HeLa-derived cDNA library a novel serine/threonine kinase (Goodpasture antigen-binding protein (GPBP)) that phosphorylates the N-terminal region of the human domain (Raya, A. Revert, F, Navarro, S. and Saus J. (1999) J. Biol. Chem. 274, 12642–12649). We show here that the pre-mRNA of GPBP is alternatively spliced in human tissues and that the most common transcript found encodes GPBPΔ26, a molecular isoform devoid of a 26-residue serine-rich motif. Recombinantly expressed GPBPΔ26 exhibits lower activity than GPBP, due at least in part to a reduced ability of GPBPΔ26 to interact and to form very active high molecular weight aggregates. In human tissues, GPBP shows a more limited expression than GPBPΔ26 but displays a remarkable preference for the small vessels and for histological structures targeted by natural autoimmune responses including alveolar and glomerular basement membranes, the two main targets in Goodpasture disease. GPBP expression is, in turn, up-regulated in the striated muscle of a Goodpasture patient and in other autoimmune conditions including cutaneous lupus erythematosus, pemphigoid, and lichen planus.
Related Links	http://www.jbc.org/content/275/51/40392.abstract
Ending Page	40399
Starting Page	40392
Page Count	8
File Format	HTM / HTML PDF
ISSN	00219258
Journal	Journal of Biological Chemistry (JBC)
Issue Number	51
Volume Number	275
DOI	10.1074/jbc.M002769200
e-ISSN	1083351X
Language	English
Publisher	American Society for Biochemistry and Molecular Biology
Publisher Date	2000-12-22
Access Restriction	Open
Subject Keyword	Goodpasture (GP) Base pair (bp) Cutaneous lupus erythematosus (CLE) Recombinant counterparts of GPBP and GPBPΔ26 (rGPBP and rGPBPΔ26) High performance liquid chromatography (HPLC) Thousand base pairs (kb) Non-collagenous C-terminal domain (NC1) Polymerase chain reaction (PCR) Reverse-transcriptase-coupled polymerase chain reaction (RT-PCR) Systemic lupus erythematosus (SLE) Polyacrylamide gel electrophoresis (PAGE) ENZYME CATALYSIS AND REGULATION
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Molecular Biology

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