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核苷酸内焦磷酸酶/磷酸二酯酶1抗体费用 英文名称  Anti-ENPP1/PC1/3

中文名称  核苷酸内焦磷酸酶/磷酸二酯酶1抗体费用 

别    名 ENPP1; ENPP 1; ENPP-1; PC1; PC-1; ectonucleotide pyrophosphatase/phosphodiesterase 1; Plasma cell membrane glycoprotein PC 1; Phosphodiesterase I/nucleotide pyrophosphatase 1; BDP; EC; NEC1; PC1; PC3; Pcsk1; Prohormone convertase 1; Prohormone convertase 1/3; Prohormone convertase 3; Proprotein convertase 1; SPC3.

产品属性：

浓    度  1mg/1ml

规 格  0.1ml/100μg 0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Rabbit

产品类型   一抗  

研究领域     免疫学 信号转导 激酶和磷酸酶

蛋白分子量  predicted molecular weight: 100kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human ENPP1 N-terminus

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

核苷酸内焦磷酸酶/磷酸二酯酶1抗体费用 产品应用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.  

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. 

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

产品介绍 ENPP1 has a broad specificity and cleaves a variety of substrates, including phosphodiester bonds of nucleotides and nucleotide sugars and pyrophosphate bonds of nucleotides and nucleotide sugars. It can hydrolyze nucleoside 5' triphosphates such as ATP, GTP, CTP, TTP and UTP to their corresponding monophosphates with release of pyrophosphate. It can also hydrolyze diadenosine polyphosphates and 3',5'-cAMP to AMP. It may play a role in the regulation of pyrophosphate production, the regulation of the availability of nucleotide sugars in the endoplasmic reticulum and Golgi, and the regulation of purinergic signaling.

The subtilisin-like Prohormone Convertase (PC) family is a group of cellular enzymes that cleave most prohormones and neuropeptide precursors. Numerous other cellular proteins, some viral proteins, and bacterial toxins that are transported by the constitutive secretory pathway are also targeted for maturation by PCs. PC family members share structural similarities, which include a heterogeneous ~10 kDa amino-terminal proregion, a highly conserved ~55 kDa subtilisin-like catalytic domain, and carboxyl-terminal domain that is heterogeneous in length and sequence. These enzymes become catalytically active following proregion cleavage within the appropriate cellular compartment. The subcellular localization of PC family members varies. Immunolocalization studies show that PC1 is found in the perinuclear region as well as the trans-Golgi network, whereas PC2 can be found in the trans-Golgi network as well as diffusely distributed in the peripheral cytoplasm.

Function : Involved in the processing of hormone and other protein precursors at sites comprised of pairs of basic amino acid residues. Substrates include POMC, renin, enkephalin, dynorphin, somatostatin and insulin.

Subunit : Homodimer; disulfide-linked.

Tissue Specificity : Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.

DISEASE : Defects in PCSK1 are the cause of proprotein convertase 1 deficiency (PC1 deficiency) [MIM:600955]. PC1 deficiency is characterized by obesity, hypogonadism, hypoadrenalism, reactive hypoglycemia as well as marked small-intestinal absorptive dysfunction It is due to impaired processing of prohormones.

Similarity : Belongs to the peptidase S8 family. Furin subfamily.

实验流程：

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