catalog number :
MBS150079
products full name :
PRICKLE1 Antibody
products short name :
PRICKLE1
products name syn :
PRICKLE1; RILP; EPM1B; RILP; Prickle-like protein 1; prickle homolog 1
other names :
prickle-like protein 1; Prickle-like protein 1; prickle-like protein 1; prickle-like 1; REST/NRSF-interacting LIM domain protein 1; REST (RE-1 silencing transcription factor)/NRSF (neuron-restrictive silencer factor)-interacting LIM domain protein; prickle homolog 1 (Drosophila); REST/NRSF-interacting LIM domain protein 1
products gene name :
PRICKLE1
other gene names :
PRICKLE1; PRICKLE1; RILP; EPM1B; RILP
uniprot entry name :
PRIC1_HUMAN
reactivity :
Human, Mouse, Rat
specificity :
Multiple isoforms of PRICKLE1 are known to exist. PRICKLE1 antibody is predicted to not cross-react with other PRICKLE protein family members.
purity :
PRICKLE1 Antibody is affinity chromatography purified via peptide column.
storage stability :
PRICKLE1 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
tested application :
ELISA (EIA), Western Blot (WB), Immunofluorescence (IF)
app notes :
PRICKLE1 antibody can be used for detection of PRICKLE1 by Western blot at 1 mug/mL. Antibody can also be used for immunofluorescence starting at 20 mug/mL. For immunofluorescence start at 20 mug/mL.
other info1 :
Conjugate: Unconjugated. Immunogen: PRICKLE1 antibody was raised against a 19 amino acid synthetic peptide near the carboxy terminus of human PRICKLE1. Buffer: PRICKLE1 Antibody is supplied in PBS containing 0.02% sodium azide.
products description :
PRICKLE1 Antibody: PRICKLE1, also known as RILP or EPM1B, is a Disheveled-associated protein that serves as a nuclear translocation receptor for REST/NRSF and REST4 (1, 2). It contains three N-terminal LIM domains and three C-terminal nuclear localization signals. It localizes to the cytoplasm, as well as to the nuclear membrane and expressed at higher levels in placenta. PRICKLE1 is a negative regulator of the Wnt / beta-catenin signaling pathway and is a putative tumor suppressor in human HCCs. Defects in the gene encoding PRICKLE1 are associated with autosomal recessive progressive myoclonic epilepsy.
ncbi gb acc num :
NM_001144883.1
ncbi mol weight :
94,300 Da
ncbi pathways :
Asymmetric Localization Of PCP Proteins Pathway (1127529); PCP/CE Pathway (1127528); Signal Transduction Pathway (477114); Signaling By Wnt Pathway (106510); Wnt Signaling Pathway (83061); Wnt Signaling Pathway (471); Beta-catenin Independent WNT Signaling Pathway (1127527)
ncbi summary :
This gene encodes a nuclear receptor that may be a negative regulator of the Wnt/beta-catenin signaling pathway. The encoded protein localizes to the nuclear membrane and has been implicated in the nuclear trafficking of the transcription repressors REST/NRSF and REST4. Mutations in this gene have been linked to progressive myoclonus epilepsy. Alternate splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 3. [provided by RefSeq, Sep 2009]
uniprot summary :
PRICKLE1: Involved in the planar cell polarity pathway that controls convergent extension during gastrulation and neural tube closure. Convergent extension is a complex morphogenetic process during which cells elongate, move mediolaterally, and intercalate between neighboring cells, leading to convergence toward the mediolateral axis and extension along the anteroposterior axis. Necessary for nuclear localization of REST. May serve as nuclear receptor. Defects in PRICKLE1 are the cause of progressive myoclonic epilepsy type 1B (EPM1B). EPM1B is an autosomal recessive disorder characterized by myoclonus that progresses in severity over time, tonic-clonic seizures and ataxia. Defects in PRICKLE1 may be a cause of susceptibility to neural tube defects (NTD). Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy. Failure of neural tube closure can occur at any level of the embryonic axis. Common NTD forms include anencephaly, myelomeningocele and spina bifida, which result from the failure of fusion in the cranial and spinal region of the neural tube. NTDs have a multifactorial etiology encompassing both genetic and environmental components. Belongs to the prickle / espinas / testin family. Protein type: Unknown function. Chromosomal Location of Human Ortholog: 12q12. Cellular Component: nuclear membrane; cytosol; nucleus. Molecular Function: protein binding; zinc ion binding. Biological Process: positive regulation of protein ubiquitination; protein import into nucleus; positive regulation of proteasomal ubiquitin-dependent protein catabolic process; neural tube closure; negative regulation of transcription, DNA-dependent. Disease: Epilepsy, Progressive Myoclonic 1b