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 hosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI 3-kinase) pathway and membrane trafficking.
Wildtype myotubularin can directly dephosphorylate PI 3-P and PI 4-P in vitro.
Thus, it decreases PI 3-P levels by down-regulating PI 3-K activity and by facilitating the degradation of PI 3-P.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a liquid PBS, 0.05% sodium azide, pH 7.2.
Specificity:Recognizes endogenous levels of Myotubularin. Species Crossreactivity: Human, mouse
Isotype:IgG
Calc Applications Abbrev:WB
Calc Crossreactivity:Hu Mo
Immunogen:Synthetic peptide corresponding to amino acids 256-3111 of Human Myotubularin.