FREM1 Antibody

中文名稱：

FREM1兔多克隆抗體
貨號：

CSB-PA686013LA01HU
規格：

￥440
圖片：
IHC image of CSB-PA686013LA01HU diluted at 1:500 and staining in paraffin-embedded human tonsil tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
其他：

產品詳情
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) FREM1 Polyclonal antibody
Uniprot No.：

Q5H8C1
基因名：

FREM1
別名：

BC037594 antibody; BNAR antibody; C9orf143 antibody; C9orf145 antibody; C9orf154 antibody; D430009N09 antibody; D630008K06 antibody; FLJ25461 antibody; FRAS1-related extracellular matrix protein 1 antibody; FREM 1 antibody; FREM1 antibody; FREM1_HUMAN antibody; Heb antibody; MOTA antibody; Protein QBRICK antibody; QBRICK antibody; RGD1306981 antibody; RP11-265B7.2 antibody; RP23-410K19.1 antibody; TILRR antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human FRAS1-related extracellular matrix protein 1 protein (1957-2082AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，FREM1 Antibody (CSB-PA686013LA01HU)，的標記方式是Non-conjugated。對于FREM1 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA686013LB01HU	FREM1 Antibody, HRP conjugated	ELISA
FITC	CSB-PA686013LC01HU	FREM1 Antibody, FITC conjugated
Biotin	CSB-PA686013LD01HU	FREM1 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:500-1:1000

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development.
基因功能參考文獻：
1. we identified genome-wide significant association involving measures of central upper lip height at 9p22 within FREM1 PMID: 28441456
2. FREM1 expression is significantly downregulated in human masticatory mucosa during wound healing PMID: 28005267
3. this report describes a patient with a phenotype compatible with Manitoba-oculo-tricho-anal syndrome in whom two novel FREM1 mutations were identified in the compound heterozygous state thus broadening the mutational spectrum of the disease. PMID: 28111185
4. Disruption of the FREM1 gene can produce a spectrum of clinical manifestations encompassing the previously described MOTA and BNAR syndromes. PMID: 23401257
5. We conclude that FREM1 plays a critical role in the development of the diaphragm and that FREM1 deficiency can cause CDH in both humans and mice. PMID: 23221805
6. The significant association of rs1552896 with an HIV-resistant phenotype, together with the expression profile of FREM1 in tissues relevant to HIV infection, suggests that FREM1 is a potentially novel candidate gene for resistance to HIV infection. PMID: 22915813
7. The location of the IBD region 16 kb from FREM1 suggests the phenotype in Manitoba oculotrichoanal syndrome patients is attributable to a variant outside of FREM1, potentially in a regulatory element. PMID: 22690109
8. FREM1 encodes a basement membrane protein of FRAS1-related extracellular matrix protein 1 which is required for epidermal adhesion during embryonic development PMID: 22876578
9. a role for TILRR in selective amplification of NF-kappaB responses through IL-1RI and suggest that the specificity is determined by changes in receptor conformation and adapter protein recruitment. PMID: 22262840
10. These data suggest that copy number variations and mutations involving FREM1 can be identified in a significant percentage of people with MC with or without midface hypoplasia. PMID: 21931569
11. Manitoba-oculo-tricho-anal (MOTA) syndrome is caused by mutations in FREM1. FREM1 deficiency ties the molecular cause of MOTA syndrome closely to the pathogenesis of Fraser syndrome. PMID: 21507892
12. TILRR, an isoform encoded by an alternatively spliced FREM1 mRNA, is an IL-1RI co-receptor that associates with the signaling receptor complex to enhance recruitment of MyD88 and control Ras-dependent amplification of NF-kappaB and inflammatory responses. PMID: 19940113
13. The phenotypic variability reported for different Frem1 mouse mutants suggests that the apparently distinct phenotype of bifid nose and anorectal and renal anomalies syndrome in humans may represent a previously unrecognized variant of Fraser syndrome. PMID: 19732862
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相關疾?。?/div>
Bifid nose, with or without anorectal and renal anomalies (BNAR); Manitoba oculotrichoanal syndrome (MOTA); Trigonocephaly 2 (TRIGNO2)
亞細胞定位：

Secreted, extracellular space, extracellular matrix, basement membrane.
蛋白家族：

FRAS1 family
數據庫鏈接：

HGNC: 23399

OMIM: 248450

KEGG: hsa:158326

STRING: 9606.ENSP00000370262

UniGene: Hs.50850

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