FBN2 Antibody

中文名稱：

FBN2兔多克隆抗體
貨號：

CSB-PA008457LA01HU
規格：

￥440
圖片：
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).
其他：

產品詳情
產品評價
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) FBN2 Polyclonal antibody
Uniprot No.：

P35556
基因名：

FBN2
別名：

CCA antibody; congenital contractural arachnodactyly (Marfanoid-like) antibody; DA9 antibody; FBN2 antibody; FBN2_HUMAN antibody; fibrillin 2 (congenital contractural arachnodactyly) antibody; Fibrillin-2 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Fibrillin-2 protein (304-484AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，FBN2 Antibody (CSB-PA008457LA01HU)，的標記方式是Non-conjugated。對于FBN2 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-PA008457LB01HU	FBN2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA008457LC01HU	FBN2 Antibody, FITC conjugated
Biotin	CSB-PA008457LD01HU	FBN2 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, IF

推薦稀釋比：

Application	Recommended Dilution
IF	1:200-1:500

Protocols：

ELISA Protocol
Immunofluorescence (IF) 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：

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.
基因功能參考文獻：
1. Case Report: femoral aneurysm in patient with FBN2 mutation. PMID: 29742989
2. 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
3. Decellularized lung scaffolds treated with FBN-2 and TN-C prior to re-epithelialization supported greater epithelial proliferation and tissue remodeling. PMID: 28662401
4. 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
5. 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
6. 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
7. 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
8. DNA sequence variation within the FBN2 gene is associated with both Achilles tendon (AT) and anterior cruciate ligament (ACL) rupture. PMID: 25429546
9. Rare and common variants in extracellular matrix gene Fibrillin 2 (FBN2) are associated with macular degeneration. PMID: 24899048
10. 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
11. Data demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibrils. PMID: 20404337
12. Data show that in wound healing and sclerotic skin diseases, a marked increase of fibrillin-2 expression was found by immunohistology. PMID: 20195245
13. 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
14. 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
15. there are distinct functions for fibrillin-2 in peripheral nerves PMID: 12429739
16. Relaxin regulates its mRNA and protein expression by human dermal fibroblasts and murine fetal skin PMID: 12590922
17. No associations of intracranial aneurysm and FBN2 were found. PMID: 12750963
18. A comprehensive genetic analysis of FBN2 was performed in patients with Marfan syndrome or Marfan-related phenotypes. PMID: 16835936
19. 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
20. Methylation of CLDN6, FBN2, RBP1, RBP4, TFPI2, and TMEFF2 in esophageal squamous cell carcinoma. PMID: 19288010
21. EGFR, fibrillin-2, P-cadherin and AP2beta as biomarkers for rhabdomyosarcoma diagnostics. PMID: 19469909
22. A novel mutation (C1425Y) in the FBN2 gene in a father and son with congenital contractural arachnodactyly is reported. PMID: 19473076
23. Examine association between FBN2 SNPs and intracranial aneurysms in Japanese cohort. PMID: 19506372
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相關疾病：

Arthrogryposis, distal, 9 (DA9); Macular degeneration, early-onset (EOMD)
亞細胞定位：

Secreted.; [Fibrillin-2]: Secreted, extracellular space, extracellular matrix.; [Placensin]: Secreted.
蛋白家族：

Fibrillin family
組織特異性：

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
數據庫鏈接：

HGNC: 3604

OMIM: 121050

KEGG: hsa:2201

STRING: 9606.ENSP00000262464

UniGene: Hs.519294

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