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Descriptor English: Fibrillin-1
Descriptor Spanish: Fibrilina-1
Descriptor fibrilina-1
Entry term(s) profibrilina 1
profibrilina-1
Scope note: Fibrilina (FBN1) que actúa como proteína de soporte estructural para las MICROFIBRILLAS. También regula la maduración de los OSTEOBLASTOS mediante el control de la concentración y disponibilidad de TGF-BETA y de PROTEÍNAS MORFOGENÉTICAS ÓSEAS. Las mutaciones del gen FBN1 se asocian al SÍNDROME DE MARFAN.
Descriptor Portuguese: Fibrilina-1
Descriptor French: Fibrilline-1
Entry term(s): Fibrillin 1
Profibrillin 1
Profibrillin-1
Tree number(s): D09.400.430.800.500
D12.776.395.341.500
D12.776.860.300.400.500
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D000071838
Scope note: 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.
Allowable Qualifiers: AD administration & dosage
AE adverse effects
AG agonists
AI antagonists & inhibitors
AN analysis
BI biosynthesis
BL blood
CF cerebrospinal fluid
CH chemistry
CL classification
CS chemical synthesis
DE drug effects
DF deficiency
EC economics
GE genetics
HI history
IM immunology
IP isolation & purification
ME metabolism
PD pharmacology
PH physiology
PK pharmacokinetics
PO poisoning
RE radiation effects
SD supply & distribution
ST standards
TO toxicity
TU therapeutic use
UL ultrastructure
UR urine
Public MeSH Note: 2017; see FIBRILLIN 1985-2016
History Note: 2017; use FIBRILLIN 1985-2016
DeCS ID: 56520
Unique ID: D000071838
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2017/01/01
Date of Entry: 2016/07/08
Revision Date: 2016/04/15
Fibrillin-1 - Preferred
Concept UI M0132396
Scope note 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.
Preferred term Fibrillin-1
Entry term(s) Fibrillin 1
Profibrillin 1 - Narrower
Concept UI M0246918
Preferred term Profibrillin 1
Entry term(s) Profibrillin-1



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