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Mouse Anti-NUP133 Recombinant Antibody (3E8) (CBMAB-N3984-WJ)

This product is a Mouse antibody that recognizes NUP133. The antibody 3E8 can be used for immunoassay techniques such as: ELISA, IHC-P, WB.
See all NUP133 antibodies
Published Data
Fig.1 Evaluation of the interaction between NUP107 and NUP133 with the use of wild-type NUP107 and its alterations.
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Fig.2 Immunofluorescence microscopy of NUP133.
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Summary

Host Animal
Mouse
Specificity
Human
Clone
3E8
Antibody Isotype
IgG2a, κ
Application
ELISA, IHC-P, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
nucleoporin 133kDa
Introduction
The nuclear envelope creates distinct nuclear and cytoplasmic compartments in eukaryotic cells. It consists of two concentric membranes perforated by nuclear pores, large protein complexes that form aqueous channels to regulate the flow of macromolecules between the nucleus and the cytoplasm. These complexes are composed of at least 100 different polypeptide subunits, many of which belong to the nucleoporin family. The nucleoporin protein encoded by this gene displays evolutionarily conserved interactions with other nucleoporins. This protein, which localizes to both sides of the nuclear pore complex at interphase, remains associated with the complex during mitosis and is targeted at early stages to the reforming nuclear envelope. This protein also localizes to kinetochores of mitotic cells. [provided by RefSeq, Jul 2008]
Entrez Gene ID
55746
UniProt ID
Q8WUM0
Alternative Names
Nucleoporin 133; 133 KDa Nucleoporin; Nucleoporin 133kDa; Nucleoporin Nup133; Nuclear Pore Complex Protein Nup133; Nucleoporin 133kD; HNUP133;
Function
Involved in poly(A)+ RNA transport. Involved in nephrogenesis (PubMed:30179222).
Biological Process
mRNA export from nucleusManual Assertion Based On ExperimentIDA:UniProtKB
Nephron developmentManual Assertion Based On ExperimentIMP:UniProtKB
Neural tube developmentIEA:Ensembl
NeurogenesisIEA:Ensembl
Nuclear pore organizationManual Assertion Based On ExperimentIMP:UniProtKB
Nucleocytoplasmic transport1 PublicationIC:ComplexPortal
Paraxial mesoderm developmentIEA:Ensembl
Poly(A)+ mRNA export from nucleusManual Assertion Based On ExperimentIBA:GO_Central
Protein import into nucleusManual Assertion Based On ExperimentIBA:GO_Central
Somite developmentIEA:Ensembl
Transcription-dependent tethering of RNA polymerase II gene DNA at nuclear peripheryManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Nucleus, nuclear pore complex
Chromosome, centromere, kinetochore
Located on both the cytoplasmic and nuclear sides of the nuclear pore (PubMed:11564755).
During mitosis, localizes to the kinetochores (PubMed:11564755).
Involvement in disease
Nephrotic syndrome 18 (NPHS18):
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS18 is an autosomal recessive, steroid-resistant progressive form with onset in the first decade of life.
Galloway-Mowat syndrome 8 (GAMOS8):
A form of Galloway-Mowat syndrome, a severe renal-neurological disease characterized by early-onset nephrotic syndrome associated with microcephaly, central nervous system abnormalities, developmental delays, and a propensity for seizures. Brain anomalies include gyration defects ranging from lissencephaly to pachygyria and polymicrogyria, and cerebellar hypoplasia. Most patients show facial dysmorphism characterized by a small, narrow forehead, large/floppy ears, deep-set eyes, hypertelorism and micrognathia. Additional variable features are visual impairment and arachnodactyly. Most patients die in early childhood. GAMOS8 inheritance is autosomal recessive.
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For research use only. Not intended for any clinical use.

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