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Rabbit Anti-LEPRE1/BF647 Conjugated antibody (bs-18222R-BF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-18222R-BF647
英文名称 Rabbit Anti-LEPRE1/BF647 Conjugated antibody
中文名称 BF647标记的生长抑制因子1抗体
别    名 GROS 1; GROS1; Growth suppressor 1; LEPRE 1; Lepre1; Leprecan 1; Leprecan; Leprecan-1; Leprecan1; Leucine proline enriched proteoglycan (leprecan) 1; Leucine proline enriched proteoglycan 1; Leucine- and proline-enriched proteoglycan 1; MGC117314; OI8; P3H1; P3H1_HUMAN; Prolyl 3 hydroxylase 1; Prolyl 3-hydroxylase 1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 81kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LEPRE1
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:
This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Three alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined. [provided by RefSeq, Aug 2011]

Function:
Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.

Subcellular Location:
Endoplasmic reticulum. Secreted > extracellular space > extracellular matrix. Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan.

Post-translational modifications:
O-glycosylated; chondroitin sulfate.

DISEASE:
Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8) [MIM:610915]. A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest.

Similarity:
Belongs to the leprecan family.
Contains 1 Fe2OG dioxygenase domain.
Contains 4 TPR repeats.

Database links:

Entrez Gene: 64175 Human

Entrez Gene: 56401 Mouse

Entrez Gene: 114200 Rat

Omim: 610339 Human

SwissProt: Q32P28 Human

SwissProt: Q3V1T4 Mouse

SwissProt: Q9R1J8 Rat

Unigene: 720014 Human

Unigene: 27961 Mouse

Unigene: 13741 Rat



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