Product Name	FREM1
Chinese Name	Extracellular matrix蛋白FREM1抗体
Alias	BC037594; BNAR; C9orf143; C9orf145; C9orf154; D430009N09; D630008K06; FLJ25461; FRAS1-related extracellular matrix protein 1; FREM 1; FREM1; FREM1_HUMAN; Heb; MOTA; Protein QBRICK; QBRICK; RGD1306981; RP11-265B7.2; RP23-410K19.1; TILRR.
Research Area	Cell biology  Developmental biology  Extracellular matrix
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Dog, Horse, )
Applications	ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	242kDa
Cellular localization	The cell membrane Extracellular matrix Secretory protein
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human FREM1: 1201-1300/2179
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	FREM1 is a 2,179 amino acid protein that contains one C-type lectin domain, one Calx-beta domain and twelve CSPG repeats. Localized to the basement membrane of embryonic epidermal cells and secreted into extracellular space, FREM1 functions as an extracellular matrix protein that is essential for epidermal adhesion during embryogenesis and may also participate in epidermal differentiation. FREM1 exists as multiple alternatively spliced isoforms and is encoded by a gene which maps to human chromosome 9. Chromosome 9 contains 145 million base pairs and comprises 4% of the human genome, encoding nearly 900 genes. Hereditary hemorrhagic telangiectasia, which is characterized by harmful vascular defects, and Familial dysautonomia, are both associated with chromosome 9. Notably, chromosome 9 encompasses the largest interferon family gene cluster. Function: Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development Subcellular Location: Secreted; extracellular space; extracellular matrix; basement membrane. Localizes at the basement membrane zone of embryonic epidermis and hair follicles. DISEASE: Defects in FREM1 are the cause of bifid nose with or without anorectal and renal anomalies (BNAR) [MIM:608980]. 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. Similarity: Belongs to the FRAS1 family. Contains 1 C-type lectin domain. Contains 1 Calx-beta domain. Contains 12 CSPG (NG2) repeats. SWISS: Q5H8C1 Gene ID: 158326 Database links: Entrez Gene: 158326 Human Entrez Gene: 329872 Mouse Omim: 608944 Human SwissProt: Q5H8C1 Human SwissProt: Q684R7 Mouse Unigene: 50850 Human Unigene: 242337 Mouse

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