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Ⅲ型胶原蛋白/胶原蛋白3/3型胶原蛋白抗体
  • 产品货号：
    BN42185R
  • 中文名称：
    Ⅲ型胶原蛋白/胶原蛋白3/3型胶原蛋白抗体
  • 英文名称：
    Rabbit anti-Collagen III Polyclonal antibody

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    产品规格

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  • BN42185R-50ul

    50ul

    ¥1486.00

    交叉反应：Human,Rat(predicted:Mouse,Cow) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

  • BN42185R-100ul

    100ul

    ¥2360.00

    交叉反应：Human,Rat(predicted:Mouse,Cow) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

  • BN42185R-200ul

    200ul

    ¥3490.00

    交叉反应：Human,Rat(predicted:Mouse,Cow) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

英文名称Collagen III
中文名称Ⅲ型胶原蛋白/胶原蛋白3/3型胶原蛋白抗体
别    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..  


研究领域细胞生物  免疫学  信号转导  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Rat,  (predicted: Mouse, Cow, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量117kDa
细胞定位细胞外基质 分泌型蛋白 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Collagen alpha 1(III) chain:801-900/1466 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍The extensive family of COL gene products (collagens) is composed of several chain types, including fibril-forming interstitial collagens (types I, II, III and V) and basement membrane collagens (type IV), each type containing multiple isoforms. Collagens are fibrous, extracellular matrix proteins with high tensile strength and are the major components of connective tissue, such as tendons and cartilage. All collagens contain a triple helix domain and frequently show lateral self-association in order to form complex connective tissues. Several collagens also play a role in cell adhesion, important for maintaining normal tissue architecture and function.

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.

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.

SWISS:
P02461

Gene ID:
1281

Database links:

Entrez Gene: 510833 Cow

Entrez Gene: 1281 Human

Entrez Gene: 12825 Mouse

Entrez Gene: 84032 Rat

Omim: 120180 Human

SwissProt: P04258 Cow

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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