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- Table of Contents
1 Citations
Facts about Paired box protein Pax-3.
Transcriptional activator of MITF, acting synergistically with SOX10 (PubMed:21965087). .
Human | |
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Gene Name: | PAX3 |
Uniprot: | P23760 |
Entrez: | 5077 |
Belongs to: |
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paired homeobox family |
CDHS; HUP2; HUP2MGC120384; MGC120381; MGC120382; MGC120383; MGC134778; paired box 3; paired box gene 3 (Waardenburg syndrome 1); paired box homeotic gene 3; paired box protein Pax-3; paired domain gene 3; paired domain gene HuP2; Pax3; Waardenburg syndrome 1; WS1; WS3
Mass (kDA):
52.968 kDA
Human | |
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Location: | 2q36.1 |
Sequence: | 2; NC_000002.12 (222199887..222298998, complement) |
Nucleus.
Are you having difficulty choosing the appropriate primary antibody for the PAX3 marker? It's not a problem for everyone. Read on to learn more about the PAX3 Marker, its Clinical applications and references. We'll review the main antibody, its Clinical applications and references. Finally, we'll talk about the Boster Bio company's PAX3 Marker, the company's PAX3 assay, and the benefits of PAX3 as biomarker.
The Anti-Pax3 Marker in the Boster Bio Library was developed using a specific antibody against Pax3. This antibody detects the polyubiquitinated and monoubiquitinated forms of Pax3. This is in contrast to the canonical views on the mechanisms of protein degradation, which include polyubiquitination as well as proteasomal cleavage. This agent is also sensitive ubiquitinated Pax3.
The Anti-Pax3 Marker in the Boster Bio antibody library was previously identified as a form of polyubiquitination of Pax3. The polyubiquitinated state was confirmed by immunoblotting of the purified protein. The Western analysis of the blot was used to study the results. The polyubiquitinated form of Pax3 is taken from its monoubiquitinated counterpart.
The level of protein in Pax3 declined as myogenic lineage cells matured. It was rare to observe Pax3+ cells in myoblast cultures that had been established. However, Pax3 transcript was highly activated during the activation of satellite cells and peaked on day three. It continued to be expressed at an intermediate level after protein levels declined. The protein levels dropped, and was eventually undetectable in myogenic progenitors.
It is not clear what differential regulation is referring to for Pax3 and Pax7. However, the two proteins are highly homologous and a common antagonist of these processes could regulate the levels of either. It is possible that the differential regulation of Pax3 and Pax7 could help in identifying the mechanism behind this. These two proteins are regulated by ubiquitinationand protein monoubiquitination and proteasomal degradation.
PAX3 is an epidermal substance that is found in the skin. It is often misinterpreted as an indicator of the aging process. In fact, PAX3 is expressed by various types of cells that include epidermal keratinocytes pigmented melanocytes, and fibroblasts. This antibody detects PAX3 expression in epidermal cells, and detects its protein coexpression with Ki67.
We utilized the PAX3 marker to identify the primary antibody. First, paraffin embedded sections were removed from the wax and blocked with 10 percent NGS. Secondary antibodies were added and incubated at 4°C for 1 hour. After that it was time to use rabbit PAX3 for the immunofluorescence used. Hoechst 334242 was the primary antibody used. The sections were mounted in FluorSave Reagent (Calbiochem) and analyzed using an Olympus BX51 microscope equipped with a DP71 camera.
The immune system also expresses PAX3 in response to melanoma cell growth. While its role in regulation isn't fully understood, it is believed to contribute to the growth and progression of certain types of tumors. Boster Bio's PAX3 antibody is a principal antibody, targets melanoma and the naevi cell. Its role in regulating the biology of melanoma cells might be connected to its role as a regulator.
In immunohistochemistry, PAX3 expression was assessed in paraffin-embedded tissue samples of normal skin, naevus, and primary melanoma. The intensity of PAX3 expression was low in normal skin and metastases of melanoma. It is unclear if PAX3 expression is present in melanocytes that are differentiating.
Three genes encode the PAX3 gene: DNA protein, and mRNA. DNA diagrams show a paired-box domain exons, exons, and a promoter. Both RNA and protein diagrams include open boxes to show functional domains and conserved regions. The protein diagram shows the dimensions of each type PAX3 gene. The antibody can detect PAX3 both in the cellular model as well as in cells.
A recent study has revealed that PAX3 is expressed in 81.6 percent of melanocytes expressing MITF in human skin. Immunohistochemistry results showed that PAX3 expression does not occur due to an increase in the number of individual cells but rather the rise in the number of cells that have this protein. The results of immunofluorescence also revealed that PAX3 was present in melanocytes of normal skin and metastases from melanoma.
The detection of PAX3 mutations in human tumors will assist to identify patients suffering from a variety of illnesses. PAX3 mutations in mice may lead to the discovery of genes that regulate PAX3 expression. In addition, identifying mutations in PAX3 proteins will allow researchers to pinpoint other genes that alter the expression of PAX3.
The PAX3 gene is made up of three parts: DNA and the mRNA. In the DNA diagram the exons are numbered and are indicated by a horizontal arrow indicating transcription direction. The mRNA diagram illustrates the start codons in vertical lines. In the diagram of proteins, conserved regions are denoted by open boxes, and functional domains are identified by thick horizontal lines above the proteins. Thin horizontal line segments represent the sizes of the proteins in a representative manner. The PAX3 protein structure consists of a homeodomain, a paired domain and a serine rich area.
Although PAX3 was not detected in the data sets, it was present in cancer cells. The inhibition of PAX3 by the mTORC1-kinase inhibitor rapamycin (PQR620) reduced the survival and proliferation of MB cells belonging to Group 3 in the laboratory. In in vivo, PAX3 expression is sufficient to reduce SOX2 levels. These results suggest that PAX3 is a good marker for cancer.
Recent gene ontogeny studies revealed that PAX3 GOF tumorspheres as well as PAX6 GOF tumorspheres had different expression of photoreceptors as well as GABAergic transmitter ion channels. Similarly, epithelial cells with PAX3 and PAX6 GOF tumorspheres were significantly deregulated in certain GABAergic and glutamatergic ion channels. These results are in support of the use of PAX3 and PAX6 tumorspheres as surrogate models to study the mechanisms that regulate MB progression.
Researchers also discovered a 14-bp deletion within the PAX3 gene, which resulted in an exon 3 premature termination codon. The gene product was incomplete and was deficient in the majority of paired domains as well as predicted homeodomains. The deletion started at codon 158 and continued through codon 162 in the paired box. Researchers reported that the deletion caused haploinsufficiency in the product protein and transdominant negative effects.
The wild-type variant of PAX3 is believed to play an important part in the development and progression of various cancers. Its function in normal development could be the reason for the wild-type role of PAX3. The function of the mutant PAX3 is unclear. The presence of a PAX3 mutation in a cancer patient's tissues should be taken into consideration when determining the significance of PAX3 expression in the tumor.
Because pax3 can be found in the unfused shelves of the Palatal shelves, the expression of PAX3 is not lost entirely at P0. In addition, it remains strongly expressed in the tongue. This indicates that the mutant version is not a determinant for leukemia, or other disorders. The PAX3 gene is found in more than a dozen tissues. PAX3 expression is maintained throughout human lifespan.
PAX3 is a member of the PAX family of transcription factors. It is expressed in numerous tissues and plays a pivotal role in the development of embryos. After birth, PAX3 is also essential in maintaining normal cell function. The gene is composed of nine exons, each with an activation domain for transcription at the C-terminus. It has approximately 100 kb of DNA. The 2;13 translocation disrupts one 18-kb intron and preserves the DNA-binding domain as well as the transcriptional activation domain.
The PAX3-FOXO1 fusion protein is much more powerful than other versions of the PAX3 gene, which results in excessively increased downstream target gene expression. It also interacts with MYOD1, MYOG, MYCN and chromatin structural proteins, like BRD4. Dysregulated target genes contribute to tumorigenesis by altering signal pathways and inhibiting gene expression.
PMID: 14639621 by Parker C.J., et al. Expression of PAX 3 alternatively spliced transcripts and identification of two new isoforms in human tumors of neural crest origin.
PMID: 7782066 by Macina R.A., et al. Genomic organization of the human PAX3 gene: DNA sequence analysis of the region disrupted in alveolar rhabdomyosarcoma.
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