Information on EC 2.7.11.9 - Goodpasture-antigen-binding protein kinase

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The expected taxonomic range for this enzyme is: Euteleostomi

EC NUMBER
COMMENTARY
2.7.11.9
-
RECOMMENDED NAME
GeneOntology No.
Goodpasture-antigen-binding protein kinase
REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
ATP + [Goodpasture antigen-binding protein] = ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
phospho group transfer
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
ATP:[Goodpasture antigen-binding protein] phosphotransferase
This serine/threonine kinase specifically binds to and phosphorylates the N-terminal region of the human Goodpasture antigen, which is located on the alpha3 chain of collagen IV and is involved in autoimmune disease.
SYNONYMS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
Collagen type IV alpha 3 binding protein
-
-
-
-
Goodpasture antigen-binding protein (ceramide transporter)
-
-
Goodpasture antigen-binding protein kinase
-
-
Goodpasture antigen-binding protein kinase
-
-
Goodpasture antigen-binding protein-1
Q9EQG9
-
goodpasture-antigen-binding protein kinase
-
-
-
-
goodpasture-antigen-binding protein kinase
Q9Y5P4
-
GPBP
-
-
-
-
GPBP
Q5M7Y0
-
GPBP
B3KUB7
-
GPBP kinase
-
-
GPBP-1
Q9EQG9
-
GPBPDELTA26
Q5M7Y0
-
GPBPDELTA26/CERT
B3KUB7
-
StAR-related lipid transfer protein 11
-
-
-
-
StARD11
-
-
-
-
START domain-containing protein 11
-
-
-
-
CAS REGISTRY NUMBER
COMMENTARY
230316-19-5
-
ORGANISM
COMMENTARY
LITERATURE
SEQUENCE CODE
SEQUENCE DB
SOURCE
-
SwissProt
Manually annotated by BRENDA team
GPBPDELTA26; gene COL4A3BP; GPBP; gene COL4A3BP
UniProt
Manually annotated by BRENDA team
gene COL4A3BP
B3KUB7
UniProt
Manually annotated by BRENDA team
isoform 1
SwissProt
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
metabolism
-
downregulation of GPBP kinase activity by human biliverdin reductase, protein-protein interactions between the two kinases, and regulation of the TNF-alpha/NFkappaB-dependent expression of GPBP by human biliverdin reductase
physiological function
-
GPBP-1 is a non-conventional kinase that regulates glomerular basement membrane collagen organization. It targets GIP130, a new myosin-binding protein, and regulates myofibrillogenesis in cultured myoblasts. GPBP-1 is a kinase for structural protein organization at both intracellular and extracellular compartments
physiological function
Q9Y5P4
Goodpasture antigen-binding protein, GPBP, is a nonconventional Ser/Thr kinase for basement membrane type IV collagen
SUBSTRATE
PRODUCT                      
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
ATP + goodpasture antigen
ADP + phosphorylated goodpasture antigen
show the reaction diagram
-, Q9Y5P4
phosphorylation in the N-terminal region of the goodpasture antigen
-
?
ATP + goodpasture antigen
ADP + phosphorylated goodpasture antigen
show the reaction diagram
-
phosphorylation in the N-terminal region of the goodpasture antigen
-
-
?
ATP + goodpasture antigen
ADP + phosphorylated goodpasture antigen
show the reaction diagram
-, Q9Y5P4
enzyme is involved in autoimmune pathogenesis. Enzyme expression is up-regulated in the striated muscle of a Goodpasture patient and in other autoimmune conditions including cutaneous lupus erythematosus, pemphigoid, and lichen planus
-
?
ATP + membrane type IV collagen
ADP + phopshorylated membrane type IV collagen
show the reaction diagram
B3KUB7
GPBP serves as transporter of ceramide between the endoplasmic reticulum and the Golgi apparatus. The 91-kDa GPBP splicing variants regulates the levels of 77-kDa GPBP variant, i.e. GPBPDELTA26/CERT, in the extracellular compartment, Goodpasture-antigen binding protein, GPBP, is a nonconventional Ser/Thr kinase for basement membrane type IV collagen. The 77-kDa GPBP is a soluble extracellular protein that interacts with type IV collagen
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
Q9Y5P4
-
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
autophosphorylation via the enzyme's serine/threonine kinase activity that specifically targets a motif in the NC1 domain of the alpha3 chain of type IV collagen, the Good pasture autoantigen, the enzyme is involved in autoimmune processes and disease, autophosphorylation via the enzyme's serine/threonine kinase activity that specifically targets a motif in the NC1 domain of the alpha3 chain of type IV collagen, the Good pasture autoantigen
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-, Q5M7Y0
the Goodpasture antigen-binding protein is an atypical protein kinase that phosphorylates the Goodpasture auto-antigen, the alpha3 chain of collagen IV
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
the Goodpasture antigen-binding protein performs autophosphorylation
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
the Goodpasture antigen-binding protein performs autophosphorylation
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
Q9Y5P4
the Goodpasture antigen-binding protein performs phosphorylation of collagen IV
-
-
?
additional information
?
-
-, Q5M7Y0
disruption of the nonvesicular ceramide transport is detrimental to normal embryonic development of somites and brain because of increased apoptosis mediated by the CERT function of GBPB, but not GBPBDELT26, overview
-
-
-
additional information
?
-
-, Q5M7Y0
isozyme GPBP also function as ceramide transfer proteins, CERT
-
-
-
additional information
?
-
-, Q5M7Y0
isozyme GPBPDELTA26 also functions as ceramide transfer protein, CERT
-
-
-
additional information
?
-
Q9Y5P4
Goodpasture antigen-binding protein binds to human serum amyloid P component specifically in its physiological conformations, i.e. pentamers and decamers. Collagen IV binds to serum amyloid P and to Goodpasture antigen-binding protein, the latter also binds laminin, binding studies and kinetics, overview
-
-
-
additional information
?
-
-
GPBP-1 targets the GPBP-interacting protein of 130 kDa, GIP130, which binds to myosin and promotes its myofibrillar assembly
-
-
-
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
ATP + goodpasture antigen
ADP + phosphorylated goodpasture antigen
show the reaction diagram
-, Q9Y5P4
enzyme is involved in autoimmune pathogenesis. Enzyme expression is up-regulated in the striated muscle of a Goodpasture patient and in other autoimmune conditions including cutaneous lupus erythematosus, pemphigoid, and lichen planus
-
?
ATP + membrane type IV collagen
ADP + phopshorylated membrane type IV collagen
show the reaction diagram
B3KUB7
GPBP serves as transporter of ceramide between the endoplasmic reticulum and the Golgi apparatus. The 91-kDa GPBP splicing variants regulates the levels of 77-kDa GPBP variant, i.e. GPBPDELTA26/CERT, in the extracellular compartment
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
autophosphorylation via the enzyme's serine/threonine kinase activity that specifically targets a motif in the NC1 domain of the alpha3 chain of type IV collagen, the Good pasture autoantigen, the enzyme is involved in autoimmune processes and disease
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-, Q5M7Y0
the Goodpasture antigen-binding protein is an atypical protein kinase that phosphorylates the Goodpasture auto-antigen, the alpha3 chain of collagen IV
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
the Goodpasture antigen-binding protein performs autophosphorylation
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
-
the Goodpasture antigen-binding protein performs autophosphorylation
-
-
?
ATP + [Goodpasture antigen-binding protein]
ADP + [Goodpasture antigen-binding phosphoprotein]
show the reaction diagram
Q9Y5P4
the Goodpasture antigen-binding protein performs phosphorylation of collagen IV
-
-
?
additional information
?
-
-, Q5M7Y0
disruption of the nonvesicular ceramide transport is detrimental to normal embryonic development of somites and brain because of increased apoptosis mediated by the CERT function of GBPB, but not GBPBDELT26, overview
-
-
-
additional information
?
-
Q9Y5P4
Goodpasture antigen-binding protein binds to human serum amyloid P component specifically in its physiological conformations, i.e. pentamers and decamers. Collagen IV binds to serum amyloid P and to Goodpasture antigen-binding protein, the latter also binds laminin, binding studies and kinetics, overview
-
-
-
additional information
?
-
-
GPBP-1 targets the GPBP-interacting protein of 130 kDa, GIP130, which binds to myosin and promotes its myofibrillar assembly
-
-
-
COFACTOR
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
ATP
-, Q5M7Y0
-
METALS and IONS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
Mg2+
-
required
additional information
Q9Y5P4
binding of collagen IV to Goodpasture antigen-binding protein is independent of the presence of Ca2+
INHIBITORS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
human biliverdin reductase
-
i.e. hBVR, inhibits GPBP kinase activity, and hBVR decreases GPBP autophosphorylation in a concentration-dependent manner. A 7-residue peptide, KKRILHC281, corresponding to the core of the consensus D(delta)-Box motif in the interacting domain of hBVR, is as effective as the intact 296-residue hBVR polypeptide in inhibiting GPBP kinase activity. The D(delta)-Box motif is a common feature of kinases in the MAPK signaling cascade
-
ACTIVATING COMPOUND
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
TNF-alpha
-
stimulates the autophosphorylation of Goodpasture antigen-binding protein
-
additional information
-
expression of the Goodpasture antigen-binding protein is induced by tumor necrosis factor
-
pH OPTIMUM
pH MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
7
-
-
assay at
SOURCE TISSUE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
SOURCE
Q9Y5P4
Goodpasture antigen-binding protein is present in brain amyloid plaques
Manually annotated by BRENDA team
-
GPBP-1 accumulates in the cytoplasm of differentiating mouse myoblasts prior to myosin synthesis
Manually annotated by BRENDA team
additional information
Q9GKI7
enzyme is preferentially expressed in small vessels and histological structures targeted by natural autoimmune responses including alveolar and glomerular basement membranes
Manually annotated by BRENDA team
additional information
-
preferential expression in the histological structures that are targets of common autoimmune responses
Manually annotated by BRENDA team
additional information
-, Q5M7Y0
isozymes GPBP and GPBPDELTA26 are differentially expressed during development, isozymes function during embryogenesis, GPBP is an important factor for normal skeletal muscle and brain development, overview; isozymes GPBP and GPBPDELTA26 are differentially expressed during development, isozymes function during embryogenesis, overview
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
B3KUB7
isozyme GPBPDELTA26/CERT
Manually annotated by BRENDA team
B3KUB7
77 kDa GPBP
-
Manually annotated by BRENDA team
B3KUB7
91 kDa GPBP
Manually annotated by BRENDA team
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
phosphoprotein
-
Goodpasture antigen-binding protein performs autophosphorylation
Purification/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
recombinant FLAG-tagged Goodpasture antigen-binding protein from Escherichia coli strain BL21(DE3) by affinity chromatography
Q9Y5P4
Cloned/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
gene COL4A3BP, located on chromosome 5, the gene product is alternatively spliced producing two protein isozymes: GPBP and GPBPDELTA26, DNA and amino acid sequence determination and analysis, expression of wild-type and mutant isozyme GPBPDELTA26 in HEK-293 cells; gene COL4A3BP, located on chromosome 5, the gene product is alternatively spliced producing two protein isozymes: GPBP and GPBPDELTA26, DNA and amino acid sequence determination and analysis, expression of wild-type and mutant isozyme GPBP in HEK-293 cells
-, Q5M7Y0
DNA sequence determination and analysis, a TNF-responsive transcription unit that locates the enzyme in the signalling cascade of TNF, regulation of transcription, e.g. by tumor necrosis factor, via cooperation in a bidirectional transcription complex with the gene encoding the DNA polymerase kappa, NFkappaB, and a 140-bp promoter with a Sp1 site and a TATA-like element, promoter analysis, overview
-
expression of FLAG-tagged Goodpasture antigen-binding protein in Escherichia coli strain BL21(DE3)
Q9Y5P4
expression of FLAG-tagged Goodpasture antigen-binding protein in HEK-293A cells
-
gene COL4A3BP, DNA and amino acid sequence determinatio and analysis, gene COL4A3BP bears an alternative canonical translation initiation site, iMet, that extends the open reading frame of the 91-kDa polypeptide upstream by 45 residues, expression of FLAG-tagged GPBP in HeLa cells, two splicing variants GPBP and GPBPDELTA26/CERT. Native GPBP polypeptides of 91 kDa and 120 kDa are expressed insolubly associated with cellular membranes, whereas native GPBPDELTA26/CERT polypeptide of 77 kDa is expressed soluble in the cytoplasm, overview
B3KUB7
EXPRESSION
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
TNF-alpha-mediated transcriptional activation of GPBP, not affected by human biliverdin reductase
-
ENGINEERING
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
N460A/Y461A
Q9Y5P4
site-directed mutagenesis, the mutant shows unaltered binding of serum amyloid P
P500G/A501Q
Q9Y5P4
site-directed mutagenesis, the mutant binding to serum amyloid P is abolished
V498A/W499A
Q9Y5P4
site-directed mutagenesis, the mutant shows unaltered binding of serum amyloid P
additional information
-, Q5M7Y0
construction of antisense oligonucleotide-mediated loss-of-function and synthetic mRNA-based gain-of-function mutants. The loss-of-function phenotype is linked to cell death, evident primarily in the muscle of the somites, extensive loss of myelinated tracks, and brain edema, phenotype, overview
I439A/W588A
Q9Y5P4
site-directed mutagenesis, the mutant shows reduced binding of serum amyloid P
additional information
Q9Y5P4
immobilization of Goodpasture antigen-binding protein by human serum amyloid P component binding from solution and in plasma, overview. Serum amyloid P associates in the blood with a 37-kDa GPBP fragment containing the N-terminal region and the middle domain
Y461A/F462A
Q9Y5P4
site-directed mutagenesis, the mutant shows reduced binding of serum amyloid P
additional information
-
gpbp-1-/- mice and Tg-hGPBP-1 animals do not display any obvious morphological phenotype, gpbp-1-/- mice also do not show a functional phenotype, whereas Tg-hGPBP-1 mice display reduced endurance that is not attributable to mitochondrial alterations