Product Name	Anti-FREM2/AF555
Chinese Name	AF555标记的Extracellular matrix蛋白FREM2抗体
Alias	DKFZp781I048; ECM3 homolog; FRAS1-related extracellular matrix protein 2; FREM 2; Frem2; FREM2_HUMAN; KIAA1074.
Research Area	Cardiovascular  Cell biology  Developmental biology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	346kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human FREM2
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: FREM2 is a 3,169 amino acid single-pass type I membrane protein that localizes to the extracellular side of the cell membrane and contains five Calx-beta domains, as well as 12 CSPG repeats. Functioning as an extracellular matrix protein, FREM2 is required for the maintenance of skin and renal epithelia and is also thought to be involved in epidermal adhesion events. Defects or mutations in the gene encoding FREM2, which maps to human chromsome 13, are associated with Fraser syndrome, a multisystem malformation that is characterized by ear abnormalities, congenital heart defects and cutaneous syndactyly. FREM2 exists as multiple alternatively spliced isoforms. Function: 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. Subcellular Location: Cell membrane. DISEASE: Defects in FREM2 are a cause of Fraser syndrome (FRASS) [MIM:219000]. Fraser syndrome is a multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects. Similarity: Belongs to the FRAS1 family. Contains 5 Calx-beta domains. Contains 12 CSPG (NG2) repeats. Database links: Entrez Gene: 341640 Human Entrez Gene: 242022 Mouse Entrez Gene: 310418 Rat Omim: 608945 Human SwissProt: Q5SZK8 Human SwissProt: Q6NVD0 Mouse Unigene: 253994 Human Unigene: 38378 Mouse Unigene: 63994 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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