Symbol
Name
ID

Fbn1
fibrillin 1
MGI:95489

Phenotype annotations related to respiratory system

!	Indicates phenotype varies with strain background.
	Darker colors indicate more annotations

Human Phenotypes	Pulmonary artery dilatation	Pulmonary arterial hypertension	Pneumothorax	Emphysema	Respiratory insufficiency
Disease(s) Associated with FBN1	Pulmonary artery dilatation	Pulmonary arterial hypertension	Pneumothorax	Emphysema	Respiratory insufficiency
geleophysic dysplasia 2
Marfan syndrome

Mouse Phenotypes

abnormal lung vasculature morphology

hemothorax

lung hemorrhage

lung inflammation

abnormal lung morphology

abnormal lung development

impaired lung alveolus development

abnormal pulmonary neuroendocrine body morphology

increased number of pulmonary neuroendocrine bodies

abnormal pulmonary alveolus morphology

overexpanded pulmonary alveolus

abnormal pulmonary interalveolar septum morphology

abnormal respiratory bronchiole morphology

emphysema

respiratory distress

decreased respiration

Availability

Mouse Genotype

Fbn1^tm1Rmz/Fbn1^tm1Rmz

Fbn1^tm2Rmz/Fbn1^tm2Rmz

Fbn1^tm3Rmz/Fbn1^tm3Rmz

Fbn1^tm1Hcd/Fbn1⁺

Fbn1^tm1Lper/Fbn1⁺

Fbn1^tm1.1Itl/Fbn1^tm3Rmz
Tg(CMV-cre)1Cgn/0 (conditional)

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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