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Rabbit Anti-AMER1 (N-term) Recombinant Antibody (CBXF-2276) (CBMAB-F2039-CQ)

This product is a rabbit antibody that recognizes AMER1 (N-term). The antibody CBXF-2276 can be used for immunoassay techniques such as: WB.
See all AMER1 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
CBXF-2276
Antibody Isotype
IgG
Application
WB

Basic Information

Immunogen
Recombinant protein specific to the amino terminus of human WTX protein.
Host Species
Rabbit
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
HEPES, pH 7.5, 0.1 Mg/ml BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
Batch dependent
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
N-term

Target

Full Name
APC Membrane Recruitment Protein 1
Introduction
The protein encoded by this gene upregulates trancriptional activation by the Wilms tumor protein and interacts with many other proteins, including CTNNB1, APC, AXIN1, and AXIN2. Defects in this gene are a cause of osteopathia striata with cranial sclerosis (OSCS).
Entrez Gene ID
139285
UniProt ID
Q5JTC6
Alternative Names
APC Membrane Recruitment Protein 1; Adenomatous Polyposis Coli Membrane Recruitment 1; Wilms Tumor Gene On The X Chromosome Protein; Family With Sequence Similarity 123B; Wilms Tumor On The X; Protein FAM123B;
Function
Regulator of the canonical Wnt signaling pathway. Acts by specifically binding phosphatidylinositol 4,5-bisphosphate (PtdIns(4,5)P2), translocating to the cell membrane and interacting with key regulators of the canonical Wnt signaling pathway, such as components of the beta-catenin destruction complex. Acts both as a positive and negative regulator of the Wnt signaling pathway, depending on the context: acts as a positive regulator by promoting LRP6 phosphorylation. Also acts as a negative regulator by acting as a scaffold protein for the beta-catenin destruction complex and promoting stabilization of Axin at the cell membrane. Promotes CTNNB1 ubiquitination and degradation. Involved in kidney development.
Biological Process
Adipose tissue development Source: Ensembl
Beta-catenin destruction complex assembly Source: Reactome
Beta-catenin destruction complex disassembly Source: Reactome
Bone development Source: Ensembl
Mesenchymal cell differentiation involved in kidney development Source: Ensembl
Negative regulation of canonical Wnt signaling pathway Source: UniProtKB
Positive regulation of canonical Wnt signaling pathway Source: UniProtKB
Positive regulation of cellular protein catabolic process Source: ParkinsonsUK-UCL
Positive regulation of protein ubiquitination Source: ParkinsonsUK-UCL
Regulation of canonical Wnt signaling pathway Source: UniProtKB
Wnt signaling pathway Source: Reactome
Cellular Location
Cytoplasm; Cell membrane; Nucleus. Shuttles between nucleus and cytoplasm. Detected in nuclear paraspeckles that are found close to splicing speckles. Translocates to the cell membrane following binding to PtdIns(4,5)P2.
Involvement in disease
Osteopathia striata with cranial sclerosis (OSCS): An X-linked dominant sclerosing bone dysplasia that presents in females with macrocephaly, cleft palate, facial palsy, conductive hearing loss, mild learning disabilities, sclerosis of the long bones and skull. Longitudinal striations are visible on radiographs of the long bones, pelvis, and scapulae (osteopathia striata). In males this entity is usually associated with fetal or neonatal lethality. Occasional surviving males have, in addition to hyperostosis, cardiac, intestinal, and genitourinary malformations.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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