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I型膠原單克隆抗體
  • 產品貨號：
    BN42047M
  • 中文名稱：
    I型膠原單克隆抗體
  • 英文名稱：
    Mouse anti-Collagen I Monoclonal antibody
  • 品牌：
    Biorigin

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    產品規(guī)格

    售價

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  • BN42047M-50ul

    50ul

    ￥1486.00

    交叉反應：Rat(predicted:Human,Mouse) 推薦應用：IHC-P,IHC-F,ICC,IF

  • BN42047M-100ul

    100ul

    ￥2360.00

    交叉反應：Rat(predicted:Human,Mouse) 推薦應用：IHC-P,IHC-F,ICC,IF

產品描述

英文名稱Collagen I
中文名稱I型膠原單克隆抗體
別    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; Collagen alpha-1(II) chain; Alpha-1 type II collagen; Collagen alpha-1(II) chain; Chondrocalcin; collagen alpha-1(I) chain preproprotein.  
研究領域細胞生物  免疫學  細胞外基質  
抗體來源Mouse
克隆類型Monoclonal
克 隆 號7C7
交叉反應Rat,  (predicted: Human, Mouse, )
產品應用IHC-P=1:100-1000 IHC-F=1:100-1000 ICC=1:50-500 IF=1:50-500 （石蠟切片需做抗原修復）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量139kDa
細胞定位細胞外基質 分泌型蛋白 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Collagen I: 
亞    型IgG
純化方法affinity purified by Protein G
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產品介紹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.

SWISS:
P02452

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.