IGHMBP2 antibody (AA 271-355) (FITC)
Quick Overview for IGHMBP2 antibody (AA 271-355) (FITC) (ABIN1393148)
Target
See all IGHMBP2 AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- AA 271-355
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Cross-Reactivity
- Mouse
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Predicted Reactivity
- Human,Rat,Cow,Sheep,Pig
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Purification
- Purified by Protein A.
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Immunogen
- KLH conjugated synthetic peptide derived from human SMUBP2
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Isotype
- IgG
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Application Notes
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 1 μg/μL
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Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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Preservative
- ProClin
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Precaution of Use
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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Storage
- -20 °C
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Storage Comment
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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Expiry Date
- 12 months
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- IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))
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Alternative Name
- SMUBP2
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Background
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Synonyms: AEP, Antreeze enhancer binding protein, ATP-dependent helicase IGHMBP2, Cardiac transcription factor 1, Cardiac transcription factor1, CATF 1, CATF1, DNA-binding protein SMUBP-2, GF-1, Glial factor 1, HCSA, HMN 6, HMN6, IGHMBP 2, Ighmbp2, Immunoglobulin mu binding protein 2, Immunoglobulin mu binding protein2, Immunoglobulin mu-binding protein 2, Immunoglobulin S mu binding protein 2, Immunoglobulin S mu binding protein2, RIPE3 b1, RIPE3b 1, RIPE3b1, SMARD 1, SMARD1, SMBP2_HUMAN, SMUBP 2.
Background: IGHMBP2 is a 993 amino acid nuclear and cytoplasmic protein that is ubiquitously expressed. Belonging to the DNA2/NAM7 helicase family, IGHMBP2 is a 5' to 3' helicase that unwinds RNA and DNA duplexes in an ATP-dependent reaction. IGHMBP2 also acts as a transcriptional regulator and is necessary for transcriptional activation of the flounder liver-type antifreeze protein gene. IGHMBP2 exists as a homooligomer and is part of the cytosolic ribonucleoprotein complex. Mutations in the gene encoding IGHMBP2 are suggested to lead to distal hereditary motor neuronopathy type 6 (HMN6), also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). HMN6 is characterized by weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs and severe respiratory distress.
Target
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