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>   首页   >   产品   >   一抗   >   其他   >   PCSK1/NEC1 Rabbit pAb   

PCSK1/NEC1 Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF
Primary Accession P29120
Other Accession P29120
Host Rabbit
Clonality Polyclonal
Calculated MW 84152 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human PCSK1/NEC1
Epitope Specificity 311-410/753
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the peptidase S8 family. Furin subfamily.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to subcellular compartments where a second autocatalytic even takes place and the catalytic activity is acquired. The protease is packaged into and activated in dense core secretory granules and expressed in the neuroendocrine system and brain. This gene encodes one of the seven basic amino acid-specific members which cleave their substrates at single or paired basic residues. It functions in the proteolytic activation of polypeptide hormones and neuropeptides precursors. Mutations in this gene have been associated with susceptibility to obesity and proprotein convertase 1/3 deficiency. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene [provided by RefSeq, Jan 2014]
Additional Information
Gene ID 5122
Other Names PC1; PC3; BMIQ12; NEC1; PC1/3; SPC3; Nec-1; Phpp-1; BDP; NEC1_BOVIN; PCSK1; NEC 1; Prohormone convertase 1; Proprotein convertase 1 (PC1); 3.4.21.93; NEC1_HUMAN; NEC1_MOUSE; Furin homolog; Propeptide-processing protease; Att-1; NEC1_RAT; proprotein convertase subtilisin/kexin type 1; neuroendocrine convertase 1; prohormone convertase 3; proprotein convertase 1/3
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name PCSK1
Synonyms NEC1
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, oxytocin, vasopressin, enkephalin, dynorphin, somatostatin, insulin and AGRP.
Cellular Location Cytoplasmic vesicle, secretory vesicle. Note=Localized in the secretion granules
Research Areas
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