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FRAS1-related extracellular matrix protein

Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development.

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FRAS1-related extracellular matrix protein 1

Also known as FRAS1-related extracellular matrix protein 1 (Protein QBRICK).
FREM1: Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development. Defects in FREM1 are the cause of bifid nose with or without anorectal and renal anomalies (BNAR). A bifid nose is a rare congenital deformity due to failure of the paired nasal processes to fuse to a single midline organ during early gestation. BNAR is an autosomal recessive disorder and patients usually present a bifid nose associated with renal and anorectal malformations. Defects in FREM1 are the cause of Manitoba oculotrichoanal syndrome (MOTA). MOTA is a rare condition defined by eyelid colobomas, cryptophthalmos, and anophthalmia/microphthalmia, an aberrant hairline, a bifid or broad nasal tip, and gastrointestinal anomalies such as omphalocele and anal stenosis. Defects in FREM1 are the cause of trigonocephaly type 2 (TRIGNO2). TRIGNO2 is a keel-shaped deformation of the forehead, caused by premature fusion of the metopic sutures. It results in a triangular shape of the head. Belongs to the FRAS1 family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 9p22.3

Disease: Bifid Nose With Or Without Anorectal And Renal Anomalies; Manitoba Oculotrichoanal Syndrome; Trigonocephaly 2

FRAS1-related extracellular matrix protein 2

Also known as FRAS1-related extracellular matrix protein 2 (ECM3 homolog).
FREM2: Extracellular matrix protein required for maintenance of the integrity of the skin epithelium and for maintenance of renal epithelia. May be required for epidermal adhesion. Defects in FREM2 are a cause of Fraser syndrome (FRASS). Fraser syndrome is a multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects. Belongs to the FRAS1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Extracellular matrix; Membrane protein, integral

Chromosomal Location of Human Ortholog: 13q13.3

Disease: Fraser Syndrome

FRAS1-related extracellular matrix protein 3

FREM3: Extracellular matrix protein which may play a role in cell adhesion. Belongs to the FRAS1 family.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 4q31.21

Cellular Component: basement membrane; extracellular matrix; extracellular space

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