抗体

兔抗人类胶原, 类型III, 甲1多克隆抗体

MBS712287

0.05毫升

375美元

多克隆

兔

未共轭

人类, 小鼠, 大鼠

免疫印迹, 酶联免疫吸附测定, 免疫细胞化学, 酶免疫法

抗体

兔抗人类胶原, 类型III, 甲1多克隆抗体

胶原, 类型III, 甲1

胶原;类型III;甲1;COL3A1;EDS4A;FLJ34534

collagen, type III, alpha 1; Collagen alpha-1(III) chain; collagen alpha-1(III) chain; collagen alpha-1(III) chain; collagen, fetal; alpha1 (III) collagen; Ehlers-Danlos syndrome type IV, autosomal dominant; collagen, type III, alpha 1

COL3A1;COL3A1;EDS4A

CO3A1_HUMAN

多克隆

IgG

兔

人类, 小鼠, 大鼠

1466

抗原亲和纯化

酶联免疫吸附测定(EIA), 免疫印迹(免疫印迹), 免疫荧光(IF)

Immunogen: Human COL3A1

Storage Buffer: PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.

270048012

ACZ58371.1

111,907 Da

Amoebiasis Pathway (167324); Amoebiasis Pathway (167191); Assembly Of Collagen Fibrils And Other Multimeric Structures Pathway (730306); Binding And Uptake Of Ligands By Scavenger Receptors Pathway (771599); Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Endothelins Pathway (137958); Extracellular Matrix Organization Pathway (576262)

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]

CO3A1: Collagen type III occurs in most soft connective tissues along with type I collagen. Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3); 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). 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). 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. Belongs to the fibrillar collagen family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Extracellular matrix; Secreted; Cell adhesion; Motility/polarity/chemotaxis; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 2q31. Cellular Component: extracellular matrix; extracellular space; endoplasmic reticulum lumen; extracellular region; collagen type III. Molecular Function: integrin binding; protein binding; metal ion binding; platelet-derived growth factor binding; extracellular matrix structural constituent; SMAD binding. Biological Process: skin development; integrin-mediated signaling pathway; platelet activation; axon guidance; receptor-mediated endocytosis; extracellular matrix organization and biogenesis; collagen fibril organization; wound healing; heart development; cell-matrix adhesion; negative regulation of immune response; positive regulation of Rho protein signal transduction; extracellular matrix disassembly; collagen catabolic process; response to radiation; gut development; response to mechanical stimulus; transforming growth factor beta receptor signaling pathway; response to cytokine stimulus; fibril organization and biogenesis; peptide cross-linking; cerebral cortex development; skeletal development; aging. Disease: Ehlers-danlos Syndrome, Type Iv, Autosomal Dominant; Ehlers-danlos Syndrome, Type Iii

0.05毫升

375美元

0.15毫升

715