bs-9178R [Primary Antibody]
MTM1 Polyclonal Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
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: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. 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.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
FCM(1:20-100)
IHC-P(1:200-400)
IF(IHC-P)(1:50-200)

Cross Reactive Species: Human
Mouse
Rat

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

VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded Rat colon; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with MTM1 Polyclonal Antibody, Unconjugated (bs-9178R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody for 90 minutes, and DAPI for nuclei staining.


Paraformaldehyde-fixed, paraffin embedded Mouse stomach; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with MTM1 Polyclonal Antibody, Unconjugated (bs-9178R at 1:500 overnight at 4°C, followed by a conjugated secondary for 20 minutes and DAB staining.


Paraformaldehyde-fixed, paraffin embedded Human cervical carcinoma; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with MTM1 Polyclonal Antibody, Unconjugated (bs-9178R ) at 1:500 overnight at 4°C, followed by a conjugated secondary for 20 minutes and DAB staining.


Lane 1: Mouse Colon; Probed with MTM1 Polyclonal Antibody, unconjugated (bs-9178R) at 1:500 overnight at 4°C followed by a conjugated secondary antibody for 60 minutes at 37°C.


Rectum lysates probed with MTM1 Polyclonal Antibody, Unconjugated (bs-79178R) at 1:1000 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.