Muscleblind-like 1 Antibody (HL-1822 3A4-1E9)

Western Blot: Muscleblind-like 1 Antibody (HL-1822 3A4-1E9) [NB110-37256] - Detection of Muscleblind in HeLa whole cell extract (33ug) using NB 110-37256.

Product Discontinued

Muscleblind-like 1 Antibody (HL-1822 3A4-1E9) Summary

• Immunogen: Recombinant human Muscleblind-like 1 protein. [UniProt# Q9NR56]
• Epitope: The epitope recognized is between amino acids 116-182.
• Localization: Nuclear
• Isotype: IgG1 Kappa
• Clonality: Monoclonal
• Host: Mouse
• Gene: MBNL1
• Purity: Tissue culture supernatant

Applications/Dilutions

• Dilutions:
  • Western Blot 1:1000-1:5000
  • Immunocytochemistry/Immunofluorescence 1:50-1:250
  • Immunohistochemistry 1:1000
  • Immunohistochemistry-Paraffin 1:1000
• Application Notes: This antibody is useful for Western blot where a band is seen at ~40 kDa, Immunofluorescence and Immunohistochemistry on paraffin-embedded sections. The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
• Theoretical MW: 42 kDa.
  Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
• Control:

  HeLa Lysate (NB800-PC1)

Reactivity Notes

Human, mouse and rat.

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: No buffer
• Preservative: 10mM Sodium Azide
• Purity: Tissue culture supernatant

Alternate Names for Muscleblind-like 1 Antibody (HL-1822 3A4-1E9)

• DKFZp686P06174
• EXP40
• EXP42
• EXPKIAA0428EXP35
• MBNL
• muscleblind (Drosophila)-like
• muscleblind-like (Drosophila)
• muscleblind-like protein 1
• Triplet-expansion RNA-binding protein

Background

pre-mRNAs splicing /removal of non-coding regions is a critical process which contributes to a diverse proteome as well as gene expression regulation, and there are several splicing factors that are exclusively involved in a subset of splicing decisions - human muscleblind-like family of RNA-binding proteins: MBNL1/2/3 also known as MBNL/EXP, MBLL/MPL1, and MBXL/CHCR, respectively. Muscleblind-like 1 (MBNL1) is a known mediator of pre-mRNA alternative splicing regulation and it acts either as activator or repressor of splicing on specific pre-mRNA targets. Several pathologies are linked to expansion of polynucleotide sequences (mostly trinucleotide repeats) and CUG/CCUG expansion RNAs accumulates in Myotonic Dystrophy patient cells wherein it contributes to the disease by dysregulating alternative splicing factors such as CUGBP, Elav-like Family Member 1 (CELF1) and MBNL1. The expanded CUG repeats sequester MBNL proteins into nuclear foci, leading to loss of active protein and the sequestration of MBNL1 leads to missplicing of developmentally regulated events that are responsible for symptoms in DM types 1 and 2, such as the missplicing of the chloride channel (CLCN1) leading to myotonia. CELF1 and MBNL1 regulate a number of common splicing events, but in opposite directions and loss of MBNL1 activity is more critical than upregulation of CELF1 activity for abnormal splicing.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for Muscleblind-like 1 Antibody (NB110-37256)(1)

Publication using NB110-37256

• Ami Mankodi, MD, Patana Teng-Umnuay, MD, PhD, Matt Krym, BSc, Don Henderson, BSc, Maurice Swanson, PhD, Charles A Thornton, MD. Ribonuclear Inclusions in Skeletal Muscle in Myotonic Dystrophy Types 1 2. Ann Neurol; 54:760. 2003 [PMID: 14681885] (ICC/IF, Human)

Review for Muscleblind-like 1 Antibody (NB110-37256) (1)

Average Rating: 4

(Based on 1 review)

We have 1 review tested in 1 species: Human.

reviewed by: Anonymous WB Human 01/24/2016

Summary

• Application: Western Blot
• Sample Tested: human lymphocytes, whole cell lysate
• Species: Human
• Lot: 012404

Blocking

• Blocking Details: 5% Milk, 1% BSA in TBS-T, 1H room temperature

Primary Anitbody

• Dilution Ratio: 1:1000 in 5% Milk, 1% BSA in TBS-T, 1H room temperature

Secondary Antibody

• Secondary Description: Amersham ECL mouse IgG, HRP-linked whole Ab (from sheep)
• Secondary Manufacturer Cat#: NA931-1ML
• Secondary Concentration: 1:5000

Details

• Detection Notes: ECL, 5 minute exposure

Comments

• Comments: 40ug of whole cell lysate loaded.

Bioinformatics

• Gene Symbol: MBNL1