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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB, ICC, IP | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MABE162 |
| Description | Anti-IGHMBP2 Antibody, clone11-24 |
| Alternate Names |
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| Background Information | Immunoglobulin 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. |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
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| Presentation | Purified mouse monoclonal IgG2bκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-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 |
|
| Application Notes | Immunofluorescence 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 | |
|---|---|
| Immunogen | Recombinant Protein corresponding to human IGHMBP2. |
| Clone | mAb11-24 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Isotype | IgG2bκ |
| Species Reactivity |
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| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Entrez Gene Summary | This 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 |
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| Purification Method | Protein G |
| UniProt Number |
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| UniProt Summary | FUNCTION: 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.) |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated 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 |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |