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Mouse Anti-Collagen III/BF350 Conjugated antibody (bsm-33129M-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bsm-33129M-BF350
英文名稱 Mouse Anti-Collagen III/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的III型膠原單克隆抗體
別    名 COL 3A1; COL3A1; Collagen alpha 1(III) chain; Collagen III alpha 1 chain precursor; Collagen III alpha 1 polypeptide; Collagen type III alpha 1 (Ehlers Danlos syndrome type IV autosomal dominant); Collagen type III alpha 1; Collagen type III alpha; EDS4A; Ehlers Danlos syndrome type IV, autosomal dominant; Fetal collagen; Type III collagen; CO3A1_HUMAN; Collagen alpha-1(III) chain; Type III collagen; type III preprocollagen alpha 1 chain.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號(hào) 7B6
交叉反應(yīng) Human,  (predicted: Mouse, Rat, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 117kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Collagen III
亞    型 IgG
純化方法 affinity purified by Protein G
儲(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
保存條件 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.
產(chǎn)品介紹 background:
The This gene encodes the pro-alpha1 chains of type III collagen, a fibrillar collagen that is found in extensible connective tissues such as skin, lung, uterus, intestine and the vascular system, frequently in association with type I collagen. Mutations in this gene are associated with Ehlers-Danlos syndrome types IV, and with aortic and arterial aneurysms. Two transcripts, resulting from the use of alternate polyadenylation signals, have been identified for this gene. [provided by R. Dalgleish, Feb 2008]

Function:
Collagen type III occurs in most soft connective tissues along with type I collagen.

Subunit:
Trimers of identical alpha 1(III) chains. The chains are linked to each other by interchain disulfide bonds. Trimers are also cross-linked via hydroxylysines.

Subcellular Location:
Secreted, extracellular space, extracellular matrix.

Post-translational modifications:
Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group.

DISEASE:
Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:130020]; also known as benign hypermobility syndrome. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS3 is a form of Ehlers-Danlos syndrome characterized by marked joint hyperextensibility without skeletal deformity.
Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4) [MIM:130050]. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS4 is the most severe form of the disease. It is characterized by the joint and dermal manifestations as in other forms of the syndrome, characteristic facial features (acrogeria) in most patients, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas.
Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA) [MIM:100070]. AAA is a common multifactorial disorder characterized by permanent dilation of the abdominal aorta, usually due to degenerative changes in the aortic wall. Histologically, AAA is characterized by signs of chronic inflammation, destructive remodeling of the extracellular matrix, and depletion of vascular smooth muscle cells.

Similarity:
Belongs to the fibrillar collagen family.
Contains 1 fibrillar collagen NC1 domain.
Contains 1 VWFC domain.

Database links:

Entrez Gene: 1281 Human

Entrez Gene: 12825 Mouse

Entrez Gene: 84032 Rat

Omim: 120180 Human

SwissProt: P02461 Human

SwissProt: P08121 Mouse

SwissProt: P13941 Rat

Unigene: 443625 Human

Unigene: 249555 Mouse

Unigene: 3247 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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