IGHMBP2 anticorps (AA 271-355) (Biotin)

N° du produit ABIN1393147

Détail du produit anti-IGHMBP2 anticorps (Biotin)

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

Épitope: AA 271-355

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp IGHMBP2 est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Réactivité croisée: Souris

Homologie: Human,Rat,Cow,Sheep,Pig

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human SMUBP2

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur IGHMBP2

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

Autre désignation: SMUBP2 (IGHMBP2 Produits)

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

Sujet:

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.