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ENPP1 Recombinant Rabbit mAb (bsm-60785R)  
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產(chǎn)品編號(hào) bsm-60785R
英文名稱 ENPP1 Recombinant Rabbit mAb
中文名稱 核苷酸內(nèi)焦磷酸酶/磷酸二酯酶1重組兔單克隆抗體
別    名 Alkaline phosphodiesterase 1; ARHR2; E-NPP 1; Ectonucleotide pyrophosphatase/phosphodiesterase 1; Ectonucleotide pyrophosphatase/phosphodiesterase family member 1; ENPP1_HUMAN; Ly 41 antigen; M6S1; Membrane component chromosome 6 surface marker 1; NPP1; NPPase; NPPS; Nucleotide pyrophosphatase; PC-1; PCA1; PDNP1; phosphodiesterase I/nucleotide pyrophosphatase 1; Plasma cell membrane glycoprotein 1; Plasma-cell membrane glycoprotein PC-1.  
抗體來(lái)源 Rabbit
克隆類型 Recombinant
克 隆 號(hào) C3F1
交叉反應(yīng) Human
產(chǎn)品應(yīng)用 IHC-P=1:50-100,IHC-F=1:50-100,IF=1:50-100
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 100 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ENPP1 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 PBS, Glycerol, BSA.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

Subcellular Location:
Cytoplasmic

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.

Post-translational modifications:
Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity. N-glycosylated. It has been suggested that the active SMB domain may be permitted considerable disulfide bond heterogeneity or variability, thus two alternate disulfide patterns based on 3D structures are described with 1 disulfide bond conserved in both.

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.

SWISS:
P22413

Gene ID:
5167

Database links:

Entrez Gene: 5167 Human

Entrez Gene: 18605 Mouse

Entrez Gene: 85496 Rat

Omim: 173335 Human

SwissProt: P22413 Human

SwissProt: P06802 Mouse

SwissProt: Q924C3 Rat

Unigene: 527295 Human

Unigene: 27254 Mouse

Unigene: 1199 Rat



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (human pancreas ); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (ENPP1) Monoclonal Antibody, Unconjugated (bsm-60785R) at 1:1000 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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