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MABE162 Anti-IGHMBP2 Antibody, clone11-24

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MABE162
100 µg  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, ICC, IPMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABE162
      DescriptionAnti-IGHMBP2 Antibody, clone11-24
      Alternate Names
      • immunoglobulin mu binding protein 2
      • DNA-binding protein SMUBP-2
      • cardiac transcription factor 1
      • Immunoglobulin mu-binding protein 2
      • Glial factor 1
      • ATP-dependent helicase IGHMBP2
      Background InformationImmunoglobulin mu-binding protein 2 (IGHMBP2) is a nucleic acid binding protein that acts on both RNA and DNA. The mature protein contains an ATP-dependent RNA/DNA helicase domain, an R3H single-stranded nucleic acid-binding domain, and a zinc finger domain. Consistent with these multiple functional domains, in vitro data suggests this protein is involved in and array of cellular functions such as transcriptional activation, DNA replications immunoglobulin class switching, pre-mRNA splicing, and translation.
      References
      Product Information
      FormatPurified
      Control
      • HEK293 cell lysate
      PresentationPurified mouse monoclonal IgG2bκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-IGHMBP2 Antibody, clone11-24 is a Mouse Monoclonal Antibody for detection of IGHMBP2 also known as immunoglobulin mu binding protein 2, DNA-binding protein SMUBP-2 & has been validated in WB, ICC & IP.
      Key Applications
      • Western Blotting
      • Immunocytochemistry
      • Immunoprecipitation
      Application NotesImmunofluorescence Analysis: A representative lot was used by an independent laboratory in IF. (de Planell-Saguer, M., et al. (2009). Hum Mol Genet. 18(12):2115-2126.)

      Immunoprecipitation Analysis: A representative lot was used by an independent laboratory in IP. (de Planell-Saguer, M., et al. (2009). Hum Mol Genet. 18(12):2115-2126.)
      Biological Information
      ImmunogenRecombinant Protein corresponding to human IGHMBP2.
      ClonemAb11-24
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      IsotypeIgG2bκ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis gene encodes a helicase superfamily member that binds a specific DNA sequence from the immunoglobulin mu chain switch region. Mutations in this gene lead to spinal muscle atrophy with respiratory distress type 1. [provided by RefSeq].
      Gene Symbol
      • GF-1
      • SMUBP2
      • SMARD1
      • HMN6
      • CATF1
      • HCSA
      • SMBP2
      • IGHMBP2
      Purification MethodProtein G
      UniProt Number
      UniProt SummaryFUNCTION: 5' to 3' helicase that unwinds RNA and DNA duplices in an ATP-dependent reaction. Acts as a transcription regulator. Required for the transcriptional activation of the flounder liver-type antifreeze protein gene. Exhibits strong binding specificity to the enhancer element B of the flounder antifreeze protein gene intron. Binds to the insulin II gene RIPE3B enhancer region. May be involved in translation (By similarity). DNA-binding protein specific to 5'-phosphorylated single-stranded guanine-rich sequence related to the immunoglobulin mu chain switch region. Preferentially binds to the 5'-GGGCT-3' motif. Interacts with tRNA-Tyr. Stimulates the transcription of the human neurotropic virus JCV.

      CATALYTIC ACTIVITY: ATP + H2O = ADP + phosphate.

      SUBUNIT STRUCTURE: Homooligomer. Interacts with RUVBL1, RUVBL2, GTF3C1 and ABT1. Is part of large cytosolic ribonucleoprotein complexes Probable. Associates with the ribosomes.

      SUBCELLULAR LOCATION: Nucleus. Cytoplasm. Cell projection › axon (By similarity). Note: Colocalizes with the traslation initiation factor EIF4G2 (By similarity).

      TISSUE SPECIFICTY: Expressed in all tissues examined.

      PTM: Phosphorylated upon DNA damage, probably by ATM or ATR.

      INVOLVEMENT IN DISEASE: Defects in IGHMBP2 are the cause of distal hereditary motor neuronopathy type 6 (HMN6) [MIM:604320]; also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. The most prominent symptoms of HMN6 are severe respiratory distress resulting from diaphragmatic paralysis with eventration shown on chest x-ray and predominant involvement of the upper limbs and distal muscles. SEQUENCE SIMILARITIES: Belongs to the DNA2/NAM7 helicase family.

      Contains 1 AN1-type zinc finger.

      Contains 1 R3H domain.
      Molecular Weight ~110 kDa observed. Band observed at ~60 kDa may be a proteolytic fragment of IGHMBP2. (de Planell-Saguer, M., et al. (2009). Hum Mol Genet. 18(12):2115-2126.)
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in Hek293 cell lysate.

      Western Blot Analysis: 0.5 µg/mL of this antibody detected IGHMBP2 on 10 µg of Hek293 cell 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
      Catalogue Number GTIN
      MABE162 04053252749254

      Documentation

      Anti-IGHMBP2 Antibody, clone11-24 SDS

      Title

      Safety Data Sheet (SDS) 

      Anti-IGHMBP2 Antibody, clone11-24 Certificates of Analysis

      TitleLot Number
      Anti-IGHMBP2, clone mAb11-24 - 2070894 2070894
      Anti-IGHMBP2, clone mAb11-24 - 2291728 2291728
      Anti-IGHMBP2, clone mAb11-24 - 3519869 3519869
      Anti-IGHMBP2, clone mAb11-24 - 3790005 3790005
      Anti-IGHMBP2, clone mAb11-24 - 3942185 3942185
      Anti-IGHMBP2, clone mAb11-24 - NRG1830309 NRG1830309
      Anti-IGHMBP2, clone mAb11-24 -2547216 2547216
      Anti-IGHMBP2, clone mAb11-24 -2559181 2559181
      Anti-IGHMBP2, clone mAb11-24 -2589333 2589333
      Anti-IGHMBP2, clone mAb11-24 -2594268 2594268