I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体
规格：1mg/1ml
英文名： Collagen I
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; Co
分子量： 95kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Collagen
交叉反应：Human, Mouse, Rat, Chicken, Dog, Cow, Sheep,
细胞定位：细胞外基质 分泌型蛋白
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 I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体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 I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体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. Gene ID: 1277 Database links: Entrez Gene: 282187 Cow Entrez Gene: 282188 Cow Entrez Gene: 1277 Human Entrez Gene: 1278 Human Entrez Gene: 12842 Mouse Entrez Gene: 12843 Mouse Entrez Gene: 29393 Rat Entrez Gene: 84352 Rat Omim: 120150 Human Omim: 120160 Human SwissProt: P02453 Cow SwissProt: P02465 Cow SwissProt: P02452 Human SwissProt: P08123 Human SwissProt: P11087 Mouse SwissProt: Q01149 Mouse SwissProt: P02454 Rat SwissProt: P02466 Rat Unigene: 172928 Human Unigene: 489142 Human Unigene: 681002 Human Unigene: 277735 Mouse Unigene: 458212 Mouse Unigene: 107239 Rat Unigene: 2953 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.
研究领域：肿瘤 细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid
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