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MABN102 Anti-ARX Antibody, clone 11F6.2

Detect ARX using this Anti-ARX Antibody, clone 11F6.2 validated for use in Western Blotting, IHC(P).

Anti-ARX Antibody, clone 11F6.2: Ficha de datos de seguridad (MSDS o SDS), certificado de análisis y de calidad (CoA y CoQ), expedientes, folletos y otros documentos disponibles.
Research Category: Neuroscience Research Sub-Category: Developmental Neuroscience Gene Symbol: ARX UniProt Number: Q96QS3
MABN102
100 µg  
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Tabla espec. clave

Species ReactivityKey ApplicationsHostFormatAntibody Type
HWB, IH(P)MPurifiedMonoclonal Antibody
Description
Catalogue NumberMABN102
DescriptionAnti-ARX Antibody, clone 11F6.2
Alternate Names
  • Homeobox protein ARX
  • Aristaless-related homeobox
Background InformationAristaless-related homeobox protein (ARX) is a transcription factor that plays an important role in the development of the brain, testis, and pancreas. The gene targets and nuclear associations of ARX are less clear, although it has been demonstrated that ARX interacts with the Pax4 promoter. However, over fifty ARX mutations have been identified and the resultant phenotypes have been well studied. ARX mutations are X-linked and lead to various syndromes including X-linked West syndrome; X-linked myoclonic epilepsy; Partington syndrome; and X-linked mental retardation.
References
Product Information
FormatPurified
Control
  • Human pancreas tissue lysate.
PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationDetect ARX using this Anti-ARX Antibody, clone 11F6.2 validated for use in Western Blotting, IHC(P).
Key Applications
  • Western Blotting
  • Immunohistochemistry (Paraffin)
Application NotesImmunohistochemistry Analysis: A 1:1,000 dilution from a reprsentative lot detected ARX in human brain tissue.
Biological Information
ImmunogenGST-tagged recombinant protein corresponding to human ARX.
Clone11F6.2
ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
HostMouse
IsotypeIgG1κ
Species Reactivity
  • Human
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Entrez Gene SummaryThis gene is a homeobox-containing gene expressed during development. The expressed protein contains two conserved domains, a C-peptide (or aristaless domain) and the prd-like class homeobox domain. It is a member of the group-II aristaless-related protein family whose members are expressed primarily in the central and/or peripheral nervous system. This gene is thought to be involved in CNS development. Mutations in this gene cause X-linked mental retardation and epilepsy.
Gene Symbol
  • ARX
Purification MethodProtein G Purified
UniProt Number
UniProt SummaryFUNCTION: 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.

SUBCELLULAR LOCATION: Nucleus.

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); 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); 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); 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). 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). 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.
Molecular Weight~58 kDa observed. An uncharacterized band may be observed at ~23 kDa in some tissue lysates.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated by Western Blot in human pancreas tissue lysate.

Western Blot Analysis: 2 µg/mL of this antibody detected ARX in 200 µg of human pancreas tissue lysate.
Usage Statement
  • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size100 µg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Número de referencia GTIN
MABN102 04053252549694

Documentation

Anti-ARX Antibody, clone 11F6.2 Ficha datos de seguridad (MSDS)

Título

Ficha técnica de seguridad del material (MSDS) 

Anti-ARX Antibody, clone 11F6.2 Certificados de análisis

CargoNúmero de lote
Anti-ARX, clone 11F6.2 - 2202479 2202479
Anti-ARX, clone 11F6.2 - 2287778 2287778
Anti-ARX, clone 11F6.2 - 2562846 2562846
Anti-ARX, clone 11F6.2 - 3270753 3270753
Anti-ARX, clone 11F6.2 - 3385841 3385841
Anti-ARX, clone 11F6.2 - 3557822 3557822
Anti-ARX, clone 11F6.2 - 3729434 3729434
Anti-ARX, clone 11F6.2 - 3878290 3878290
Anti-ARX, clone 11F6.2 - 3934812 3934812
Anti-ARX, clone 11F6.2 - 4027747 4027747
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New Products: Volume 3, 2012