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FIBRILLIN-1
Descriptors Found:
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DeCS
Descriptor
English
:
Fibrillin-1
Descriptor
Spanish
:
Fibrilina-1
Descriptor
Portuguese
:
Fibrilina-1
Synonyms
English
:
Profibrillin 1
Profibrillin-1
Fibrillin 1
Tree Number:
D09.400.430.875.500
D12.776.395.341.500
D12.776.860.300.400.500
Definition
English
:
A
fibrillin
(FBN1) that functions as a structural support protein for
MICROFIBRILS
. It also regulates the maturation of
OSTEOBLASTS
by controlling the availability and concentration of TGF-BETA and
BONE MORPHOGENETIC PROTEINS
.
Mutations
in the FBN1 gene are associated with
MARFAN SYNDROME.
History Note
English
:
2017; use FIBRILLIN 1985-2016
Allowable Qualifiers
English
:
AD
administration & dosage
AE
adverse effects
AG
agonists
AN
analysis
AI
antagonists & inhibitors
BI
biosynthesis
BL
blood
CF
cerebrospinal fluid
CS
chemical synthesis
CH
chemistry
CL
classification
CT
contraindications
DF
deficiency
DE
drug effects
EC
economics
GE
genetics
HI
history
IM
immunology
IP
isolation & purification
ME
metabolism
PK
pharmacokinetics
PD
pharmacology
PH
physiology
PO
poisoning
RE
radiation effects
SE
secretion
ST
standards
SD
supply & distribution
TU
therapeutic use
TO
toxicity
UL
ultrastructure
UR
urine
Record Number:
56520
Unique Identifier:
D000071838
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS