Paired Box Protein Pax-3 (PAX3) Antibody

Este producto es parte de PAX - Paired box protein Pax
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195€ (20 µl)

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935106861
info@markelab.com
name
Paired Box Protein Pax-3 (PAX3) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx001407
tested applications
ELISA, WB, IHC, IF/ICC

Description

PAX3 Antibody is a Rabbit Polyclonal antibody against PAX3. This gene is a member of the paired box (PAX) family of transcription factors. Members of the PAX family typically contain a paired box domain and a paired-type homeodomain. These genes play critical roles during fetal development. Mutations in paired box gene 3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma. The translocation t(2;13)(q35;q14), which represents a fusion between PAX3 and the forkhead gene, is a frequent finding in alveolar rhabdomyosarcoma. Alternative splicing results in transcripts encoding isoforms with different C-termini.

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Product specifications

CategoryPrimary Antibodies
Immunogen TargetPaired Box Protein Pax-3 (PAX3)
HostRabbit
ReactivityHuman, Mouse, Rat
Recommended DilutionELISA: 1 µg/ml, WB: 1/500 - 1/1000, IHC-P: 1/50 - 1/200, IF/ICC: 1/50 - 1/200. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
ClonalityPolyclonal
ConjugationUnconjugated
IsotypeIgG
PurificationPurified by affinity chromatography.
Size 120 µl
Size 2100 µl
Size 32 × 100 µl
FormLiquid
Tested ApplicationsELISA, WB, IHC, IF/ICC
BufferPBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol.
AvailabilityShipped within 5-10 working days.
StorageAliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry IceNo
UniProt IDP23760
Gene ID5077
NCBI AccessionNP_852122.1
AliasCDHS,HUP2,PAX-3,WS1,WS3
BackgroundAntibody anti-PAX3
StatusRUO
NoteConcentration: 1.58 mg/ml -

Descripción

PAX3 is a transcription factor belonging to the paired box (PAX) family, essential for embryonic development, particularly in neural crest cell migration, muscle formation, and melanocyte differentiation. PAX3 regulates genes involved in cell survival, proliferation, and lineage determination during development of the neural tube, skeletal muscles, and craniofacial structures. Mutations in PAX3 are associated with Waardenburg syndrome types I and III, characterized by hearing loss, pigmentation abnormalities, and skeletal defects due to impaired neural crest development. PAX3 also plays a role in myogenesis by activating MyoD and other myogenic regulatory factors. In cancers like alveolar rhabdomyosarcoma, chromosomal translocations involving PAX3 result in oncogenic fusion proteins (e.g., PAX3-FOXO1) that drive tumorigenesis by promoting unchecked proliferation and inhibiting differentiation. Knockout models show defects in neural crest migration, craniofacial anomalies, and muscle development failures, emphasizing PAX3's role in embryonic tissue patterning and organogenesis.

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Paired Box Protein Pax-3 (PAX3) Antibody

PAX3 Antibody is a Rabbit Polyclonal antibody against PAX3. This gene is a member of the paired box (PAX) family of transcription factors. Members of the PAX family typically contain a paired box domain and a paired-type homeodomain. These genes play critical roles during fetal development. Mutations in paired box gene 3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma. The translocation t(2;13)(q35;q14), which represents a fusion between PAX3 and the forkhead gene, is a frequent finding in alveolar rhabdomyosarcoma. Alternative splicing results in transcripts encoding isoforms with different C-termini.

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