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COMPLEMENT C1 INHIBITOR PROTEIN
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DeCS
Descriptor
English
:
Complement C1 Inhibitor Protein
Descriptor
Spanish
:
Proteína Inhibidora del Complemento C1
Descriptor
Portuguese
:
Proteína Inibidora do Complemento C1
Tree Number:
D12.776.124.486.274.920.250.500
D12.776.872.140.500
Definition
English
:
An endogenous
serine
protease inhibitor (SERPINS). It is a 105-kDa
plasma
glycoprotein, encoded by C1NH gene and produced primarily by the
liver
and
monocytes
. It inhibits a broad spectrum of proteases, including the
COMPLEMENT C1R
and the
COMPLEMENT C1S
proteases of the CLASSICAL COMPLEMENT PATHWAY, and the
MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES
. C1-INH-deficient individuals suffer from HEREDITARY
ANGIOEDEMA
.
Indexing Annotation
English
:
do not confuse with
COMPLEMENT C1 INACTIVATOR PROTEINS
Pharmacological Action:
Complement Inactivating Agents
Entry Combination - descr/qualif
English
:
Complement C1 Inhibitor Protein
/
deficiency
use
Angioedema, Hereditary
History Note
English
:
2006; for C1 ESTERASE INHIBITOR use COMPLEMENT C1 INACTIVATOR PROTEINS
Allowable Qualifiers
English
:
administration & dosage
adverse effects
agonists
analysis
biosynthesis
cerebrospinal fluid
chemistry
classification
chemical synthesis
contraindications
drug effects
diagnostic use
economics
genetics
history
immunology
isolation & purification
metabolism
pharmacology
physiology
pharmacokinetics
poisoning
radiation effects
supply & distribution
secretion
standards
toxicity
therapeutic use
ultrastructure
urine
Record Number:
50836
Unique Identifier:
D050718
Occurrence in VHL
:
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