MTM1 antibody (AA 225-275) (AbBy Fluor® 488)

Catalog No. ABIN1401286

This Rabbit Polyclonal antibody specifically detects MTM1 in WB, FACS and IF (p). It exhibits reactivity toward Human, Mouse and Rat.

Quick Overview for MTM1 antibody (AA 225-275) (AbBy Fluor® 488) (ABIN1401286)

Target: MTM1 (Myotubularin 1 (MTM1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This MTM1 antibody is conjugated to AbBy Fluor® 488

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

Product Details anti-MTM1 Antibody (AbBy Fluor® 488)

Binding Specificity: AA 225-275

Cross-Reactivity: Human, Mouse, Rat

Purification: Purified by Protein A.

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

Isotype: IgG

Application Details

Application Notes: FCM 1:20-100
IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Handling

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.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for MTM1

Target: MTM1 (Myotubularin 1 (MTM1))

Alternative Name: MTM1

Background:

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.

Gene ID: 4534

UniProt: Q13496

Pathways: Inositol Metabolic Process, Skeletal Muscle Fiber Development