Application | Comment | Organism |
---|---|---|
medicine | patients with the specific mutation K404E have a unifying syndrome in which hematological disorders predominate, the so called harderoporphyria, harderoporphyric patients exhibit iron overload secondary to dyserythropoiesis | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
mutant enzyme cloned into pGEX-2T and expressed in Escherichia coli | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
D400R | less than 1% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
F395G | 4% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
F405G | 1% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
G402A | less than 1% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
K404E | 66% of residual activity compared with wild-type CPO, causes substantial accumulation of harderoporphyrinogen | Homo sapiens |
K404N | 61% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
R401D | 45% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
R401K | 63% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
R401W | 75% of residual activity compared with wild-type CPO, causes substantial accumulation of harderoporphyrinogen | Homo sapiens |
T403N | 31% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
Y399L | 81% of residual activity compared with wild-type CPO, shows accumulation of coproporphyrinogen | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
coproporphyrinogen-III + O2 + 2 H+ | - |
Homo sapiens | protoporphyrinogen-IX + 2 CO2 + 2 H2O | - |
? | |
harderoporphyrinogen + O2 | - |
Homo sapiens | protoporphyrinogen IX + CO2 + H2O2 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
coproporphyrinogen III oxidase | - |
Homo sapiens |
CPO | - |
Homo sapiens |