Overview

  • Product name

  • Description

    Rabbit polyclonal to Arx
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Recombinant fragment corresponding to Human Arx aa 381-460.
    Sequence:

    AKWRKREKAGAQTHPPGLPFPGPLSATHPLSPYLDASPFPPHHPALDSAW TAAAAAAAAAFPSLPPPPGSASLPPSGAPL


    Database link: Q96QS3

  • Positive control

    • WB: Jurkat and HL-60 whole cell lysate; Mouse and rat brain tissue lysate. IHC-P: Human brain tissue.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.4
    Preservative: 0.03% Proclin
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Protein G purified
  • Purification notes

    Purity >95%
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab235060 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/20 - 1/200.
WB 1/500 - 1/5000. Predicted molecular weight: 58 kDa.

Target

  • Function

    Transcription factor required for normal brain development. May be important for maintenance of specific neuronal subtypes in the cerebral cortex and axonal guidance in the floor plate.
  • Tissue specificity

    Expressed predominantly in fetal and adult brain and skeletal muscle. Expression is specific to the telencephalon and ventral thalamus. There is an absence of expression in the cerebellum throughout development and also in adult.
  • Involvement in disease

    Defects in ARX are the cause of lissencephaly X-linked type 2 (LISX2) [MIM:300215]; also known as lissencephaly X-linked with ambiguous genitalia (XLAG). LISX2 is a classic type lissencephaly associated with abnormal genitalia. LISX2 patients have severe congenital or postnatal microcephaly, lissencephaly, agenesis of the corpus callosum, neonatal-onset intractable epilepsy, poor temperature regulation, chronic diarrhea, and ambiguous or underdeveloped genitalia.
    Defects in ARX are the cause of epileptic encephalopathy early infantile type 1 (EIEE1) [MIM:308350]; also known as myoclonic epilepsy X-linked with intellectual disability and spasticity, X-linked West syndrome or X-linked infantile spasm syndrome (ISSX). EIEE1 is a severe form of epilepsy characterized by frequent tonic seizures or spasms beginning in infancy with a specific EEG finding of suppression-burst patterns, characterized by high-voltage bursts alternating with almost flat suppression phases. Patients may progress to West syndrome, which is characterized by tonic spasms with clustering, arrest of psychomotor development, and hypsarrhythmia on EEG.
    Defects in ARX are a cause of Partington syndrome (PRTS) [MIM:309510]; also known as X-linked syndromic mental retardation 1 (MRXS1). PRTS is characterized by mental retardation, episodic dystonic hand movements, and dysarthria.
    Defects in ARX are the cause of mental retardation X-linked ARX-related (MRXARX) [MIM:300419]. Mental retardation is a mental disorder characterized by significantly sub-average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period.
    Defects in ARX are the cause of agenesis of the corpus callosum with abnormal genitalia (ACCAG) [MIM:300004]. A X-linked syndrome with variable expression in females. It is characterized by agenesis of corpus callosum, mental retardation and seizures. Manifestations in surviving males include severe acquired micrencephaly, mental retardation, limb contractures, scoliosis, tapered fingers with hyperconvex nails, a characteristic face with large eyes, prominent supraorbital ridges, synophrys, optic atrophy, broad alveolar ridges, and seizures. Urologic anomalies include renal dysplasia, cryptorchidism, and hypospadias.
  • Sequence similarities

    Belongs to the paired homeobox family. Bicoid subfamily.
    Contains 1 homeobox DNA-binding domain.
  • Cellular localization

    Nucleus.
  • Information by UniProt
  • Database links

  • Alternative names

    • Aristaless related homeobox gene antibody
    • Aristaless-related homeobox antibody
    • Arx antibody
    • ARX_HUMAN antibody
    • Homeobox protein ARX antibody
    • ISSX antibody
    • MRX29 antibody
    • MRX32 antibody
    • MRX33 antibody
    • MRX36 antibody
    • MRX38 antibody
    • MRX43 antibody
    • MRX54 antibody
    • MRXS1 antibody
    • phox2a antibody
    • PRTS antibody
    see all

Images

  • Paraffin-embedded human brain tissue stained for Arx using ab235060 at 1/100 dilution in immunohistochemical analysis.

    After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized tissue using an HRP conjugated SP system.

  • All lanes : Anti-Arx antibody (ab235060) at 1/500 dilution

    Lane 1 : Jurkat (human T cell leukemia cell line from peripheral blood) whole cell lysate
    Lane 2 : HL-60 (human promyelocytic leukemia cell line) whole cell lysate
    Lane 3 : Mouse brain tissue lysate
    Lane 4 : Rat brain tissue lysate.

    Secondary
    All lanes : Polyclonal Goat anti-rabbit IgG at 1/50000 dilution

    Predicted band size: 58 kDa

References

ab235060 has not yet been referenced specifically in any publications.

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