IGHMBP2 antibody (AA 271-355)

Catalog No. ABIN1386574

Product Details anti-IGHMBP2 Antibody

Target: IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))

Binding Specificity: AA 271-355

Reactivity: Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This IGHMBP2 antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC)

Cross-Reactivity: Mouse

Predicted Reactivity: Human,Rat,Cow,Sheep,Pig

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human SMUBP2

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for IGHMBP2

Target: IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))

Alternative Name: SMUBP2 (IGHMBP2 Products)

Synonyms: NV12364 antibody, CATF1 antibody, HCSA antibody, HMN6 antibody, SMARD1 antibody, SMUBP2 antibody, ZFAND7 antibody, AEP antibody, Catf1 antibody, RIPE3b1 antibody, Smbp-2 antibody, Smbp2 antibody, Smubp2 antibody, nmd antibody, sma antibody, RIP-1 antibody, RIP1 antibody, immunoglobulin mu binding protein 2 antibody, IGHMBP2 antibody, Ighmbp2 antibody, ighmbp2 antibody

Background:

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.