FBN2 Antibody
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中文名称:FBN2兔多克隆抗体
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货号:CSB-PA008457LA01HU
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规格:¥440
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促销:
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图片:
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Immunofluorescence staining of U251 cells with CSB-PA008457LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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其他:
产品详情
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产品名称:Rabbit anti-Homo sapiens (Human) FBN2 Polyclonal antibody
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Uniprot No.:P35556
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基因名:
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别名:CCA antibody; congenital contractural arachnodactyly (Marfanoid-like) antibody; DA9 antibody; FBN2 antibody; FBN2_HUMAN antibody; fibrillin 2 (congenital contractural arachnodactyly) antibody; Fibrillin-2 antibody
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宿主:Rabbit
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反应种属:Human
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免疫原:Recombinant Human Fibrillin-2 protein (304-484AA)
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免疫原种属:Homo sapiens (Human)
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标记方式:Non-conjugated
本页面中的产品,FBN2 Antibody (CSB-PA008457LA01HU),的标记方式是Non-conjugated。对于FBN2 Antibody,我们还提供其他标记。见下表:
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克隆类型:Polyclonal
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抗体亚型:IgG
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纯化方式:>95%, Protein G purified
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
产品提供形式:Liquid
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应用范围:ELISA, IF
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推荐稀释比:
Application Recommended Dilution IF 1:200-1:500 -
Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期: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.
相关产品
靶点详情
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功能:Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.; Hormone secreted by trophoblasts that promotes trophoblast invasiveness. Has glucogenic activity: is able to increase plasma glucose levels.
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基因功能参考文献:
- Case Report: femoral aneurysm in patient with FBN2 mutation. PMID: 29742989
- Sequencing analysis showed a novel missense mutation in exon 30 of FBN2 gene [c.3973G>A, p.Asp1325Asn; Chr5 (g.127670862C>T) according to NM_001999.3] PMID: 29864108
- Decellularized lung scaffolds treated with FBN-2 and TN-C prior to re-epithelialization supported greater epithelial proliferation and tissue remodeling. PMID: 28662401
- BAV patients have an increased FBN (especially FBN2) gene expression level in the ascending aorta, irrespective of dilatation, whereas MMP expression does not change significantly. PMID: 27634926
- A novel missense mutation, c.3769T>C (p.C1257R) in FBN2 was identified responsible for the genetic cause in a family with congenital contractural arachnodactyly. PMID: 27196565
- Study described a novel mutation seen in a family with three generations of congenital contractural arachnodactyly (CCA). Whole exome sequencing in two affected individuals identified a novel missense mutation in the FBN2 gene in all affected family members. PMID: 28379158
- The presence of the splice site mutation in FBN2 gene has been confirmed in a Japanese family with congenital contractural arachnodactyly complicated with aortic dilatation and dissection. PMID: 25975422
- DNA sequence variation within the FBN2 gene is associated with both Achilles tendon (AT) and anterior cruciate ligament (ACL) rupture. PMID: 25429546
- Rare and common variants in extracellular matrix gene Fibrillin 2 (FBN2) are associated with macular degeneration. PMID: 24899048
- There was no association of overall methylation of FBN2 in the serum DNA with age, maximal tumor size, extent of tumor, tumor site, histology, presence of lymph node metastasis, distant metastasis, or Dukes' stage. PMID: 23060561
- Data demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibrils. PMID: 20404337
- Data show that in wound healing and sclerotic skin diseases, a marked increase of fibrillin-2 expression was found by immunohistology. PMID: 20195245
- Ten novel mutations have been identified in the critical region of FBN2, indicating a mutation detection rate of 75% in this limited region; none of the mutations altered amino acids in the calcium binding consensus sequence of EGF-like domains. PMID: 11754102
- fibrillins can directly interact in an N- to C-terminal fashion to form homotypic fibrillin-1 or heterotypic fibrillin-1/fibrillin-2 microfibrils PMID: 12399449
- there are distinct functions for fibrillin-2 in peripheral nerves PMID: 12429739
- Relaxin regulates its mRNA and protein expression by human dermal fibroblasts and murine fetal skin PMID: 12590922
- No associations of intracranial aneurysm and FBN2 were found. PMID: 12750963
- A comprehensive genetic analysis of FBN2 was performed in patients with Marfan syndrome or Marfan-related phenotypes. PMID: 16835936
- In 14 probands, 13 new and one previously described FBN2 mutation including a mutation in exon 17, expanding the region in which FBN2 mutations occur in CCA. PMID: 19006240
- Methylation of CLDN6, FBN2, RBP1, RBP4, TFPI2, and TMEFF2 in esophageal squamous cell carcinoma. PMID: 19288010
- EGFR, fibrillin-2, P-cadherin and AP2beta as biomarkers for rhabdomyosarcoma diagnostics. PMID: 19469909
- A novel mutation (C1425Y) in the FBN2 gene in a father and son with congenital contractural arachnodactyly is reported. PMID: 19473076
- Examine association between FBN2 SNPs and intracranial aneurysms in Japanese cohort. PMID: 19506372
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相关疾病:Arthrogryposis, distal, 9 (DA9); Macular degeneration, early-onset (EOMD)
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亚细胞定位:Secreted.; [Fibrillin-2]: Secreted, extracellular space, extracellular matrix.; [Placensin]: Secreted.
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蛋白家族:Fibrillin family
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组织特异性:Almost exclusively expressed in placenta. Expressed at much lower level in other tissues. Expressed in fetal eye (18 weeks)in the retinal pigment epithelium (RPE), the choroid, Bruch's membrane and in the sclera. Not expressed in the neural retina.; [Plac
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数据库链接:
HGNC: 3604
OMIM: 121050
KEGG: hsa:2201
STRING: 9606.ENSP00000262464
UniGene: Hs.519294
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