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merck millipore,默克密理博,MAB1913,Anti-Procollagen Type I Antibody, CT, clone PCIDG10
产品名称:Anti-Procollagen Type I Antibody, CT, clone PCIDG10
产品型号:MAB1913
This Anti-Procollagen Type I Antibody, C-terminus, clone PCIDG10 is validated for use in IH for the detection of Procollagen Type I.
merck millipore,默克密理博,MAB1913,Anti-Procollagen Type I Antibody, CT, clone PCIDG10
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H, B, Gp IHC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB1913 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Procollagen Type I Antibody, CT, clone PCIDG10 产品信息 格式 Ascites 演示 Liquid. No preservative. 应用 应用 This Anti-Procollagen Type I Antibody, C-terminus, clone PCIDG10 is validated for use in IH for the detection of Procollagen Type I. 主要应用 - Immunohistochemistry
应用说明 Immunohistochemistry on paraffin embedded tissue sections fixed in acid ETOH, not traditional formalin. (1% HOAC in 95% ETOH) fixation. Suggested starting dilution 1:100 (10-25 μg/mL).
Optimal working dilutions must be determined by end user.生物信息 免疫原品种 Human pro-collagen I. 表位 C-terminus 克隆 PCIDG10 宿主 Mouse 特异性 Labels carboxy-terminal pro-peptide of collagen type I. Does not stain mature collagen fibers in tissue, but rather is localized intracellularly in cells producing pro-collagen I. Positive reaction: human, guinea pig, bovine. Negative reaction: mouse, rat, hamster. 同种型 IgG1 品种反应性 HumanBovineGuinea Pig 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000088.3
Entrez基因汇总 This gene encodes the pro-alpha1 chains of type I collagen whose triple helix comprises two alpha1 chains and one alpha2 chain. Type I is a fibril-forming collagen found in most connective tissues and is abundant in bone, cornea, dermis and tendon. Mutations in this gene are associated with osteogenesis imperfecta types I-IV, Ehlers-Danlos syndrome type VIIA, Ehlers-Danlos syndrome Classical type, Caffey Disease 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. Two transcripts, resulting from the use of alternate polyadenylation signals, have been identified for this gene. [provided by R. Dalgleish] 基因符号 - OI4
- COL1A1
非反应性品种 - Rat
- Hamster
- Mouse
UniProt编号 - P02452
UniProt汇总 FUNCTION: SwissProt: P02452 # Type I collagen is a member of group I collagen (fibrillar forming collagen).
SIZE: 1464 amino acids; 138911 Da
SUBUNIT: Trimers of one alpha 2(I) and two alpha 1(I) chains. Interacts with MRC2 (By similarity).
SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix (By similarity).
TISSUE SPECIFICITY: Forms the fibrils of tendon, ligaments and bones. In bones the fibrils are mineralized with calcium hydroxyapatite.
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: P02452 # Defects in COL1A1 are the cause of Caffey disease [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 I (EDS-I) [MIM:130000]; also known as Ehlers-Danlos syndrome gravis. Ehlers-Danlos syndrome is a genetically and phenotypically heterogeneous connective-tissue disorder characterized by loose- jointedness and fragile, velvety, stretchable, bruisable skin that heals with peculiar 'cigarette-paper' scars. EDS-I is an autosomal dominant trait. & Defects in COL1A1 are a cause of autosomal dominant Ehlers-Danlos syndrome type VII (EDS-VII) [MIM:130060]; which includes also Ehlers-Danlos syndrome type VII-A1. EDS-VII is characterized by arthrochalasis multiplex congenita, skin hyperextensibility and bruisability. & Defects in COL1A1 are a cause of osteogenesis imperfecta type I (OI-I) [MIM:166200]. OI-I is a dominantly inherited serious newborn disease characterized by bone fragility, normal stature, little or no deformity, blue sclerae and hearing loss in 50% of families. Dentinogenesis imperfecta is rare and may distinguish a subset of OI type I (formation of dentine). & Defects in COL1A1 are a cause of osteogenesis imperfecta type II (OI-II) [MIM:166210]; also known as osteogenesis imperfecta congenita. OI-II is lethal in the perinatal period and is charaterized by calvarial mineralization, beaded ribs, compressed femurs, marked long bone deformity and platyspondyly (congenital flattening of the vertebral bodies). & Defects in COL1A1 are a cause of osteogenesis imperfecta type III (OI-III) [MIM:259420]; also called progressively deforming osteogenesis imperfecta with normal sclerae. OI-III is characterized by progressively deforming bones, usually with moderate deformity at birth, sclerae is variable in color, dentinogenesis imperfecta and hearing loss are common. The stature is very short. & Defects in COL1A1 are a cause of osteogenesis imperfecta type IV (OI-IV) [MIM:166220]. OI-IV is charaterized by normal sclerae, moderate to mild deformity and variable short stature. Dentinogenesis imperfecta is common and hearing loss occurs in some patients. & Genetic variations in COL1A1 are associated with susceptibility to involutional osteoporosis [MIM:166710]; also known as senile osteoporosis or postmenopausal osteoporosis. Osteoporosis is characterized by reduced bone mineral density, disrutption of bone microarchitecture, and the alteration of the amount and variety of non-collagenous proteins in bone. Osteoporotic bones are more at risk of fracture. & A chromosomal aberration involving COL1A1 is a cause of dermatofibrosarcoma protuberans (DFSP) [MIM:607907]. Translocation t(17;22)(q22;q13) with PDGF. DFSP is an uncommon, locally aggressive, but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It typically occurs during early or middle adult life and is most frequently located on the trunk and proximal extremities.
SIMILARITY: SwissProt: P02452 ## Belongs to the fibrillar collagen family. & Contains 1 VWFC domain.产品使用声明 使用声明 - 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.
储存和货运信息 存储条件 Maintain at -20°C in aliquots up to 12 months. Avoid repeated freeze/thaw cycles. 包装信息 数量 100 µL
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