Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:1.16.3.1 (
ceruloplasmin
)
5,074
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Serum
caeruloplasmin
deficiency is a characteristic biochemical abnormality found in patients with Wilson's disease, but the mechanism of this disease is unknown. Although the phenylenediamine oxidase activity of serum
caeruloplasmin
is markedly low in patients with Wilson's disease, mRNA of
caeruloplasmin
exists to some extent. To investigate the deficiency of
caeruloplasmin
oxidase activity in Wilson's disease, we generated 14 monoclonal antibodies (MAbs) and selected
ID1
, which had the strongest reactivity, and ID2, which had neutralizing ability. We also established a system to measure active
caeruloplasmin
specifically using these MAbs. These MAbs and the system will be useful tools in analyzing the active site of
caeruloplasmin
in patients with Wilson's disease.
...
PMID:Monoclonal antibody against the active site of caeruloplasmin and the ELISA system detecting active caeruloplasmin. 751 80
Recently it was found that the clinical features of the LEC rat closely resemble those of human Wilson's disease. One of the characteristics of the animal is low levels of serum
ceruloplasmin
. Therefore, by using LEC rats, we attempted to define molecular basis of the deficiency in active site of
ceruloplasmin
in Wilson's disease patients. We made 3 monoclonal antibodies, ID2 against active site of
ceruloplasmin
,
ID1
against inactive site of
ceruloplasmin
, and the remaining one against metallothionein. Using these monoclonal antibodies, we examined immunohistochemical stainings of LEC rat liver tissues, and compared them with those of LEA rats, as a control.
ID1
stained the hepatocytes of both LEA and LEC rats, whereas ID2 stained LEA rat hepatocytes only. The results indicated that the
ceruloplasmin
secreted by LEC rat hepatocytes is mostly in inactive form. The antibody against metallothionein stained LEA rat hepatocytes only. This finding may also indicate that LEC rat hepatocytes express less amount of metallothionein than those of LEA rats.
...
PMID:Lack of copper binding sites in ceruloplasmin of LEC rats with abnormal copper metabolism. 828 Jan 28
The present study was carried out to investigate the effect of gold (Au) injection on copper (Cu) and two types of
ceruloplasmin
(Cp), total Cp (
ID1
) and active Cp (ID2), metallothionein (MT) in the serum, kidney and liver, and 8-hydroxydeoxyguanosine (8-OHdG) in the rat kidney. The Cu contents in sera and kidneys of Au-injected rats were 1.7 and 5.5 times higher than those in sera and kidneys of control rats, respectively. The most of Cu in the sera of the control rats or Au-injected rats were observed in the Cp fractions from a Sephacryl S-200 column. The Cu concentration in the Cp fractions was increased by Au injection. Significant increases of
ID1
and ID2 were found in the sera of the control rats and Au-injected rats, while there was no significant difference in those concentrations of livers or kidneys between the control rats and Au-injected rats. Our results indicated that the most of Cp existed as active
ID1
. The immunoreactivity of 8-OHdG was located in the cortex of the Au-injected rat. These results indicated that the oxidative DNA damage occurred in the renal cortex of the Au-injected rat and the localization of DNA damage did not coincide with that of Cu-MT. These findings suggest that the oxidative DNA damage in the kidneys of rats injected with Au is associated with Cu except Cu-MT.
...
PMID:The effect of Au injection on the ceruloplasmin, metallothionein and 8-hydroxydeoxyguanosine of rat serum, kidney and liver. 1220 81
