bs-9178R-Cy5

DATASHEET

Host: Rabbit

Target Protein: MTM1

Immunogen Range: 201-300/603

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 4534

Swiss Prot: Q13496

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

Purification: Purified by Protein A.

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

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

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.

Conjugation: Cy5

Excitation/ Emission: 625,650nm/670nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
FCM(1:20-100)
IF(IHC-P)(1:50-200)

Cross Reactive Species: Human
Mouse
Rat

For research use only. Not intended for diagnostic or therapeutic use.