Entity Details

Primary name FREM2
Entity type gene
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Details

PrimaryID341640
RefseqGeneNG_008125
SymbolFREM2
NameFRAS1 related extracellular matrix 2
Chromosome13
Location13q13.3
TaxID9606
Statuslive
SourceGenomegenomic
SourceOriginnatural
CreationDate2003-03-28
ModificationDate2021-06-11

Ontological Relatives

UniProt IDsFREM2_HUMAN

GO terms

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GOName
GO:0001654 eye development
GO:0001822 kidney development
GO:0002009 morphogenesis of an epithelium
GO:0005604 basement membrane
GO:0005886 plasma membrane
GO:0007154 cell communication
GO:0007155 cell adhesion
GO:0007507 heart development
GO:0016021 integral component of membrane
GO:0042733 embryonic digit morphogenesis
GO:0046872 metal ion binding
GO:0048839 inner ear development
GO:0070062 extracellular exosome

Diseases

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Disease IDSourceNameDescription
617666 OMIMFraser syndrome 2 (FRASRS2)A form of Fraser syndrome, an autosomal recessive disorder characterized by cryptophthalmos, cutaneous syndactyly, and urogenital abnormalities including renal agenesis or hypoplasia. Additional features include abnormalities of the larynx, ear malformations, and facial abnormalities. The disease is caused by variants affecting the gene represented in this entry.
123570 OMIMCryptophthalmos, unilateral or bilateral, isolated (CRYPTOP)An autosomal dominant, rare condition characterized by congenital eyelid malformation with an underlying malformed eye. It can be bilateral or unilateral and is classified into complete (typical), incomplete (atypical) and abortive (congenital symblepharon) forms. The skin of patients with complete cryptophthalmos extends uninterrupted from the forehead to the cheek, whereas incomplete cryptophthalmos exists when there is medial eyelid fusion, but coincident intact lateral structures. The symblepharon variety presents with fusion of the upper eyelid skin to the superior aspect of the globe. The complete variety is the most common form. The disease is caused by variants affecting the gene represented in this entry.