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: UNIPROT:P01034 (
cystatin C
)
3,397
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The isolated amyloid substance in hereditary
cystatin C
amyloid angiopathy (HCCAA) is mainly composed of a
cystatin C
variant devoid of the 10 amino terminal amino acid residues of extracellular
cystatin C
from healthy individuals. We have developed a procedure for protein sequencing directly from agarose gel electropherograms and used this in conjunction with isoelectric focusing to investigate the amino terminal sequence of cerebrospinal fluid (CSF)
cystatin C
in HCCAA patients. The amino-terminal sequence determined for
cystatin C
from a HCCAA patient CSF sample, Xaa-Ser-
Pro-Gly
-Lys-Pro-Pro-Xaa-Leu-Val-Gly-Gly-Pro-Met-Xaa-Ala-Xaa-Val, showed that the protein was not amino-terminally truncated. CSF
cystatin C
from all nine HCCAA patients investigated was found to have an isoelectric point identical to that of native
cystatin C
, and the truncated form of
cystatin C
isolated from amyloid deposits was shown to contribute to less than 1% of the total amount of
cystatin C
in CSF. The total cysteine proteinase inhibitory capacity of CSF from HCCAA patients was lower than that of CSF from other patients. This decreased CSF inhibitory capacity in HCCAA patients was caused by decreased levels of
cystatin C
, since the levels of the other two cysteine proteinase inhibitors found in CSF, alpha 2-macroglobulin and kininogen, were significantly higher than in CSF from non-HCCAA patients.
...
PMID:The amino terminal portion of cerebrospinal fluid cystatin C in hereditary cystatin C amyloid angiopathy is not truncated: direct sequence analysis from agarose gel electropherograms. 231 47
