英文名称    Procollagen type I N terminal propeptide    

中文名称    1型前胶原氨基末端前肽/I型前胶原氨基末端前肽抗体抗体    

别    名    COL1A1; Alpha 1 type I collagen; Alpha-1 type I collagen; CO1A1_HUMAN; COL1A1; Collagen alpha-1(I) chain; Collagen type I alpha 1; Procollagen type I N terminal propeptide; Procollagen type I N-terminal propeptide.    

1型前胶原氨基末端前肽/I型前胶原氨基末端前肽抗体抗体 

说 明 书    0.1ml  0.2ml      

研究领域    信号转导  细胞骨架  细胞外基质      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Cow, Horse, Sheep,     

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.    

分 子 量    15kDa    

细胞定位    细胞外基质 分泌型蛋白     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Procollagen type I N terminal propeptide    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

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.    

1型前胶原氨基末端前肽/I型前胶原氨基末端前肽抗体抗体 产品介绍    background:
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, Feb 2008].

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.

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.

Gene ID:
1277

Database links:

Entrez Gene: 1277 Human

Omim: 120150 Human

SwissProt: P02452 Human

Unigene: 172928 Human

Unigene: 681002 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.    

1型前胶原氨基末端前肽/I型前胶原氨基末端前肽抗体抗体 

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hz-2638R PROK1/PRK1/EG-VEGF  内分泌腺衍生血管内皮生长因子抗体

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hz-1586R VEGF-C  血管内皮生长因子C型抗体

hz-4572R VEGF-A  血管内皮生长因子A

hz-1915R VEGF-B  血管内皮生长因子B抗体

hz-2742R VEGF-D  血管内皮生长因子D型抗体

hz-0170R VEGFR1/FLT1  血管内皮生长因子受体1抗体

hz-0565R VEGFR2  血管内皮生长因子受体2抗体

hz-2674R phospho-VEGFR2(Tyr951)  磷酸化血管内皮生长因子受体2抗体

hz-3467R phospho-VEGF receptor 2 (Tyr1059)  磷酸化血管内皮生长因子受体2抗体

hz-3468R phospho-VEGFR2 (Tyr1175)  磷酸化血管内皮生长因子受体2抗体

hz-3469R phospho-VEGF receptor 2(Tyr1214)  磷酸化血管内皮生长因子受体2抗体

hz-3470R phospho-VEGF receptor 2 (Tyr996)  磷酸化血管内皮生长因子受体2抗体

hz-2089R sVEGFR2  可溶性血管内皮生长因子受体2抗体

hz-1083R VEGFR3  血管内皮细胞生长因子受体3抗体

hz-2202R VEGFR3  血管内皮细胞生长因子受体3抗体

hz-2533R Versican  蛋白聚糖Versican抗体

hz-3810R Villin  绒毛蛋白抗体

hz-0756R Vimentin  波形蛋白抗体

hz-3471R Phospho-Vimentin (Ser56)  磷酸化波形蛋白抗体

hz-3472R Phospho-Vimentin(Ser82)  磷酸化波形蛋白抗体

hz-0077R VIP  血管活性肠肽抗体

hz-3915R Vitamin D Binding protein  维生素D结合蛋白抗体

hz-0272R PBEF1(CT)  内脂素/内脏脂肪素(C端抗体)

hz-0270R PBEF(NT)  内脂素/内脏脂肪素抗体（N端）

hz-0273R PBEF (CT)  内脂素/前B细胞克隆增强因子-2（C端抗体）

hz-1931R TRPV1/VR1  辣椒素受体抗体

hz-0479R VSIG4  T淋巴细胞负调节蛋白抗体

hz-1932R VTN/Vitronectin  玻璃粘连蛋白抗体

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hz-1949R WEE1 protein  WEE1蛋白抗体