Overview

  • Product nameAnti-Arx antibody
    See all Arx primary antibodies
  • Description
    Mouse monoclonal to Arx
  • Tested applicationsWB, ELISAmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant full length human Arx protein (NP_620689) with a proprietary tag.

  • Positive control
    • SJCRH30 cell lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab89178 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
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 58 kDa.
ELISA Use at an assay dependent dilution.

Target

  • FunctionTranscription 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 specificityExpressed 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 diseaseDefects 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 similaritiesBelongs to the paired homeobox family. Bicoid subfamily.
    Contains 1 homeobox DNA-binding domain.
  • Cellular localizationNucleus.
  • 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

Anti-Arx antibody images

  • Anti-Arx antibody (ab89178) at 5 µg/ml + tagged, recombinant human Arx protein (the immunogen) at 0.2 µg

    Predicted band size : 58 kDa
    Observed band size : ~40 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 30 kDa,35 kDa. We are unsure as to the identity of these extra bands.
  • Anti-Arx antibody (ab89178) at 5 µg/ml + SJCRH30 cell lysate at 50 µg

    Predicted band size : 58 kDa
    Observed band size : 58 kDa

References for Anti-Arx antibody (ab89178)

ab89178 has not yet been referenced specifically in any publications.

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