Cell Biology Antibodies 7

Anti-FREM1 Antibody (CAB16603)

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SKU:
CAB16603
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-FREM1 Antibody
Antibody SKU:CAB16603
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1880-2179 of human FREM1 (NP_659403.4).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:293T, K-562, HepG2, Mouse kidney, Mouse liver, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1880-2179 of human FREM1 (NP_659403.4).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:GSSS STTS GSFH LERR PLPS SMQL AVIR GDTL RGFD STDL SQRK LRTR GNGK TVRP SSVY RNGT DIIY NYHG IVSL KLED DSFP THKR KAKV SIIS QPQK TIKV AELP QADK VEST TDSH FPRQ DQLP SFPK NCTL ELKG LFHF EEGI QKLY QCNG IAWK AWSP QTKD VEDK SCPA GWHQ HSGY CHIL ITEQ KGTW NAAA QACR EQYL GNLV TVFS RQHM RWLW DIGG RKSF WIGL NDQV HAGH WEWI GGEP VAFT NGRR GPSQ RSKL GKSC VLVQ RQGK WQTK DCRR AKPH NYVC SRKL
Gene ID:158326
Uniprot:Q5H8C1
Cellular Location:Secreted, basement membrane, extracellular matrix, extracellular space
Calculated MW:25kDa/42kDa/80kDa/244kDa
Observed MW:72kDa
Synonyms:FREM1, BNAR, C9orf143, C9orf145, C9orf154, MOTA, TILRR, TRIGNO2
Background:This gene encodes a basement membrane protein that may play a role in craniofacial and renal development. Mutations in this gene have been associated with bifid nose with or without anorectal and renal anomalies. Alternatively spliced transcript variants encoding different isoforms have been described. PubMed ID 19940113 describes one such variant that initiates transcription within a distinct, internal exon; the resulting shorter isoform (named Toll-like/interleukin-1 receptor regulator, TILRR) is suggested to be a co-receptor of the interleukin 1 receptor family and may regulate receptor function and Toll-like receptor/interleukin 1 receptor signal transduction, contributing to the control of inflammatory response activation.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 9p22.3

Cellular Component: integral to membrane; basement membrane

Molecular Function:metal ion binding; carbohydrate binding

Biological Process: cell-matrix adhesion; cell communication

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

NCBI Summary:This gene encodes a basement membrane protein that may play a role in craniofacial and renal development. Mutations in this gene have been associated with bifid nose with or without anorectal and renal anomalies. Alternatively spliced transcript variants encoding different isoforms have been described. PubMed ID 19940113 describes one such variant that initiates transcription within a distinct, internal exon; the resulting shorter isoform (named Toll-like/interleukin-1 receptor regulator, TILRR) is suggested to be a co-receptor of the interleukin 1 receptor family and may regulate receptor function and Toll-like receptor/interleukin 1 receptor signal transduction, contributing to the control of inflammatory response activation. [provided by RefSeq, Apr 2011]
UniProt Code:Q5H8C1
NCBI GenInfo Identifier:122056683
NCBI Gene ID:158326
NCBI Accession:NP_659403
UniProt Secondary Accession:Q5H8C1,Q684R7,
UniProt Related Accession:Q5H8C1
Molecular Weight:25,008 Da
NCBI Full Name:FRAS1-related extracellular matrix protein 1 isoform 1
NCBI Synonym Full Names:FRAS1 related extracellular matrix 1
NCBI Official Symbol:FREM1  
NCBI Official Synonym Symbols:BNAR; MOTA; TILRR; TRIGNO2; C9orf143; C9orf145; C9orf154  
NCBI Protein Information:FRAS1-related extracellular matrix protein 1; extracellular matrix protein QBRICK
UniProt Protein Name:FRAS1-related extracellular matrix protein 1
UniProt Synonym Protein Names:Protein QBRICK
Protein Family:FRAS1-related extracellular matrix protein
UniProt Gene Name:FREM1  
UniProt Entry Name:FREM1_HUMAN
Anti-FREM1 Antibody (CAB16603)Western blot analysis of extracts of various cell lines, using FREM1 antibody (CAB16603) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
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