FITC标记的I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体
产品名称： FITC标记的I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体
英文名称： Anti-Collagen I/FITC
产品编号： HZ-7158R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围：
上海沪震实业有限公司
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Rabbit Anti-Collagen I/FITC Conjugated antibody
| FITC标记的I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体 |
| 英文名称 | Anti-Collagen I/FITC |
| 中文名称 | FITC标记的I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体 |
| 别 名 | Collagen type I; Alpha 1 type I collagen; Alpha 2 type I collagen; COL1A1; COL1A2; Collagen I alpha 1 polypeptide; Collagen I alpha 2 polypeptide; Collagen Of Skin Tendon And Bone; Collagen Type 1; Collagen type I alpha 1; Collagen type I alpha 2; OI4; Osteogenesis Imperfecta Type IV; Pro alpha 1(I) collagen; Type I procollagen; CO1A1_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, |
| 产品应用 | not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 95kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Collagen alpha-1(I) chain |
| 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 | 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. |
| 产品介绍 | background: Collagens are highly conserved throughout evolution and are characterised by an uninterrupted "Glycine X Y" triplet repeat that is a necessary part of the triple helical structure. Type I collagen (95 kDa) is found in bone, cornea, skin and tendon. Mutations in the encoding gene are associated with osteogenesis imperfecta, Ehlers Danlos syndrome, and idiopathic osteoporosis. Reciprocal translocations between chromosomes 17 and 22, where this gene and the gene for Platelet-derived growth factor beta are located, are associated with a particular type of skin tumor called dermatofibrosarcoma protuberans, resulting from unregulated expression of the growth factor. Function: Type I collagen is a member of group I collagen (fibrillar forming collagen). Subunit: Trimers of one alpha 2(I) and two alpha 1(I) chains. Interacts with MRC2. Interacts with TRAM2. Subcellular Location : Secreted, extracellular space, extracellular matrix. Subcellular Location: Secreted, extracellular space, extracellular matrix. Tissue Specificity: Forms the fibrils of tendon, ligaments and bones. In bones the fibrils are mineralized with calcium hydroxyapatite. Post-translational modifications: Proline residues at the third position of the tripeptide repeating unit (G-X-P) are hydroxylated in some or all of the chains. Proline residues at the second position of the tripeptide repeating unit (G-P-X) are hydroxylated in some 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 COL1A1 are the cause of Caffey disease (CAFFD) [MIM:114000]; also known as infantile cortical hyperostosis. Caffey disease is characterized by an infantile episode of massive subperiosteal new bone formation that typically involves the diaphyses of the long bones, mandible, and clavicles. The involved bones may also appear inflamed, with painful swelling and systemic fever often accompanying the illness. The bone changes usually begin before 5 months of age and resolve before 2 years of age. Defects in COL1A1 are a cause of Ehlers-Danlos syndrome type 1 (EDS1) [MIM:130000]; also known as Ehlers-Danlos syndrome gravis. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS1 is the severe form of classic Ehlers-Danlos syndrome. Defects in COL1A1 are the cause of Ehlers-Danlos syndrome type 7A (EDS7A) [MIM:130060]; also known as autosomal dominant Ehlers-Danlos syndrome type VII. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS7A is marked by bilateral congenital hip dislocation, hyperlaxity of the joints, and recurrent partial dislocations. Defects in COL1A1 are a cause of osteogenesis imperfecta type 1 (OI1) [MIM:166200]. A dominantly inherited connective tissue disorder characterized by bone fragility and blue sclerae. Osteogenesis imperfecta type 1 is non-deforming with normal height or mild short stature, and no dentinogenesis imperfecta. Defects in COL1A1 are a cause of osteogenesis imperfecta type 2 (OI2) [MIM:166210]; also known as osteogenesis imperfecta congenita. A connective tissue disorder characterized by bone fragility, with many perinatal fractures, severe bowing of long bones, undermineralization, and death in the perinatal period due to respiratory insufficiency. Defects in COL1A1 are a cause of osteogenesis imperfecta type 3 (OI3) [MIM:259420]. A connective tissue disorder characterized by progressively deforming bones, very short stature, a triangular face, severe scoliosis, grayish sclera, and dentinogenesis imperfecta. Defects in COL1A1 are a cause of osteogenesis imperfecta type 4 (OI4) [MIM:166220]; also known as osteogenesis imperfecta with normal sclerae. A connective tissue disorder characterized by moderately short stature, mild to moderate scoliosis, grayish or white sclera and dentinogenesis imperfecta. Genetic variations in COL1A1 are a cause of susceptibility to osteoporosis (OSTEOP) [MIM:166710]; also known as involutional or senile osteoporosis or postmenopausal osteoporosis. Osteoporosis is characterized by reduced bone mass, disruption of bone microarchitecture without alteration in the composition of bone. Osteoporotic bones are more at risk of fracture. Note=A chromosomal aberration involving COL1A1 is found in dermatofibrosarcoma protuberans. Translocation t(17;22)(q22;q13) with PDGF. Similarity: Belongs to the fibrillar collagen family. Contains 1 fibrillar collagen NC1 domain. Contains 1 VWFC domain. Database links: Entrez Gene: 1277 Human·Entrez Gene: 12842 Mouse ·Entrez Gene: 100008952 Rabbit ·Entrez Gene: 29393 Rat ·Omim: 120150 Human ·SwissProt: P02453 Cow ·SwissProt: O46392 Dog ·SwissProt: P02452 Human ·SwissProt: P11087 Mouse ·SwissProt: P02454 Rat ·Unigene: 172928 Human ·Unigene: 277735 Mouse ·Unigene: 107239 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
胶原在整个进化过程中高度保守，并且具有不间断的“甘氨酸X Y”三重态重复的特征，它是三重螺旋结构的必要部分。I型胶原（95 kDa）存在于骨骼、角膜、皮肤和肌腱中。编码基因的突变与成骨不全、埃勒斯-丹洛斯综合征和特发性骨质疏松症有关。染色体17和22之间的相互易位，即该基因和血小板衍生生长因子β的基因所处的位置，与一种称为隆起性皮肤纤维肉瘤的特定类型的皮肤肿瘤相关，这种皮肤肿瘤是由于生长因子表达失控引起的。