Cloned (Comment) | Organism |
---|---|
recombinant enzyme expression of wild-type enzyme mutant enzymes in HuH-7 cells, also expression of the wild-type enzyme in Hep-G2 cells, HEK-293 cells, K-562 cells, CHO cells, and COS-1 cells, functional co-expression with the human transferrin receptor 1 substrate | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
C699A | site-directed mutagenesis, the mutant activity with substrate transferrin receptor 1 is unaltered compared to the wild-type enzyme | Homo sapiens |
C704A | site-directed mutagenesis, the mutant activity with substrate transferrin receptor 1 is unaltered compared to the wild-type enzyme | Homo sapiens |
D188G | site-directed mutagenesis, the autocatalytic processing of the PC7 zymogen is severely impaired leading to accumulation of the inactive pro-enzyme | Homo sapiens |
additional information | generation of a frameshift fsL506P enzyme mutant. Construction of an enzyme PC7 chimera, in which the transmembrane domain and the cytosolic tail are replaced by that of the convertase furin, the mutant loses its ability to cleave the transferrin receptor. Identification of the KTECER100;LA cleavage site by site-directed mutagenesis | Homo sapiens |
R316C | site-directed mutagenesis, the autocatalytic processing of the PC7 zymogen is severely impaired leading to accumulation of the inactive pro-enzyme | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
decanoyl-RVKR-chloromethylketone | - |
Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
endosome | - |
Homo sapiens | 5768 | - |
membrane | a transmembrane protein | Homo sapiens | 16020 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
human transferrin receptor 1 + H2O | Homo sapiens | cleavage at an atypical site KTECER-/-LA, in which the P1 Arg100 and P6 Lys95 are critical,.cleavage at Arg100 | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q16549 | - |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
lipoprotein | the enzyme has palmitoylated cysteines, Cys699 and Cys704 within its C-terminus, these posttranslational modifications are not required for transferrin receptor 1 shedding | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
hepatocyte | primary | Homo sapiens | - |
hepatoma cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
human transferrin receptor 1 + H2O | cleavage at an atypical site KTECER-/-LA, in which the P1 Arg100 and P6 Lys95 are critical,.cleavage at Arg100 | Homo sapiens | ? | - |
? | |
human transferrin receptor 1 + H2O | cleavage at an atypical site KTECER-/-LA, in which the P1 Arg100 and P6 Lys95 are critical, cleavage at Arg100. Identification of the KTECER100;LA cleavage site by site-directed mutagenesis using substrate mutants, overview | Homo sapiens | ? | - |
? | |
additional information | no activity with hepcidin | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
PC7 | - |
Homo sapiens |
proprotein convertase 7 | - |
Homo sapiens |
Organism | Comment | Expression |
---|---|---|
Homo sapiens | depletion of iron in the medium of hepatoma cell lines incubated with the iron chelator desferrioxamine results in the enzyme downregulation | down |
General Information | Comment | Organism |
---|---|---|
malfunction | an enzyme PC7 chimera, in which the transmembrane domain and the cytosolic tail are replaced by that of the convertase furin, loses its ability to cleave the transferrin receptor | Homo sapiens |
metabolism | when iron is limiting, human transferrin receptor TfR1 levels increase at least in part by way of the down-regulation of PC7 enzyme expression | Homo sapiens |
physiological function | the enzyme is involved in the regulation of systemic iron homeostasis via shedding of human transferrin receptor 1, TfR1, as its unique mechanism. Shedding of hTfR1 by the enzyme requires endocytosis into acidic clathrin-coated vesicles, enzyme transmembrane domain and cytosolic tail are critical for hTfR1 shedding in the endosomes | Homo sapiens |