货号：MAB1343

品牌：Merck Millipore

规格：EA

目录价：询价

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Collagen Type III Antibody, clone 4B11.1 | MAB1343 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Human type III collagen. View All» Clone: 4B11.1 View All» Isotype: IgG1 View All» Species Reactivity: Human View All» Application Notes: ELISA (antigen coated on plate): 1:64,000. Immunohistochemistry on frozen sections (air dried overnight): 1:200. Optimal working dilutions must be determined by end user. View All» Presentation: Ascites containing sodium azide. View All» Storage Conditions: Maintain at -20°C in undiluted aliquots for up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles. View All» UniProt Number: P02461 View All» Entrez Gene Number: NM_000090.3 View All» Gene Symbol:

• COL3A1

• EDS4A

• FLJ34534
  View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
  • ELISA

  • Immunohistochemistry
    View All» Entrez Gene Summary: 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] View All» UniProt Summary: FUNCTION: SwissProt: P02461 # Collagen type III occurs in most soft connective tissues along with type I collagen.SIZE: 1466 amino acids; 138564 Da 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 (By similarity).PTM: 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: SwissProt: P02461 # Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type III (EDS-III) [MIM:130020]; also known as benign hypermobility syndrome. Inheritance is autosomal dominant. EDS is characterized by joint laxity and hyperextensible skin. It is divided into nine different subtypes based on biochemical and clinical variations. & Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type IV (EDS-IV) [MIM:130050]. EDS-IV is the most severe form of the disease, in that it often produces life-threatening consequences, such as rupture of the arteries, bowel, or uterus. A variant form of EDS-IV is Gottron type acrogeria [MIM:201200]. The main characteristics are atrophy and mottled-type hyperpigmentation of the acral skin, resulting in an aged appearance. & Defects in COL3A1 may be a cause of aortic aneurysm [MIM:100070]. Aortic aneurysm consists of a dangerous ballooning of the aorta which is caused by a defect in the artery's wall.SIMILARITY: SwissProt: P02461 ## Belongs to the fibrillar collagen family. & Contains 1 VWFC domain. View All» Brand Family: Chemicon View All» Product Name: Anti-Collagen Type III Antibody, clone 4B11.1 | MAB1343 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Mouse View All»
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