MTM1 anticorps (AA 225-275) (AbBy Fluor® 647)

N° du produit ABIN1401288

Détail du produit anti-MTM1 anticorps (AbBy Fluor® 647)

Antigène: MTM1 (Myotubularin 1 (MTM1))

Épitope: AA 225-275

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp MTM1 est conjugé à/à la AbBy Fluor® 647

Application: Western Blotting (WB), Flow Cytometry (FACS), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Réactivité croisée: Humain, Souris, Rat

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human MTM1/Myotubularin

Isotype: IgG

Information d'application

Indications d'application: FCM 1:20-100
IF(IHC-P) 1:50-200

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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur MTM1

Antigène: MTM1 (Myotubularin 1 (MTM1))

Autre désignation: MTM1 (MTM1 Produits)

Synonymes: anticorps CNM, anticorps MTMX, anticorps XLMTM, anticorps wu:fb19c01, anticorps zgc:123266, anticorps myotubularin 1, anticorps myotubularin 1 S homeolog, anticorps MTM1, anticorps mtm1, anticorps mtm1.S, anticorps Mtm1

Sujet:

Synonyms: CG2, CNM, KIAA4176, mKIAA4176, Mtm, Mtm1, MTM1_HUMAN, MTMX, Myotubular myopathy 1, Myotubularin, XLMTM.

Background: X-linked recessive myotubular myopathy is a congenital muscular disease characterized by severe hypotonia and generalized muscle weakness that, in most cases, leads to early postnatal death. The gene responsible for myotubular myopathy MTM1 encodes a dual specificity phosphatase, named myotubularin, which is highly conserved through evolution. The gene for MTM1 is localized to a 300 kb critical region on human Xq128 between IDS and GRBRA3. Human MTM1, a 603 amino-acid protein, is mutated in myotubular myopathy. The largely related protein hMTMR2 is found mutated in a recessive form of Charcot-Marie-Tooth neuropathy. Myotubularin is primarily a lipid phosphatase that acts on phosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI3-kinase) pathway and membrane trafficking. Wild-type myotubularin can directly dephosphorylate PI3P and PI4P in vitro. Thus, it decreases PI3P levels by down-regulating PI3K activity and by facilitating the degradation of PI3P.

ID gène: 4534

UniProt: Q13496

Pathways: Inositol Metabolic Process, Skeletal Muscle Fiber Development