BD-PT2952

Myotubularin Rabbit Polyclonal Antibody

MTM1; CG2; Myotubularin

常规抗体

MTM1

Myotubularin

WB

Human,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

4534

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4534

Q13496

http://www.uniprot.org/uniprotkb/Q13496/entry

17772

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=17772

Q9Z2C5

http://www.uniprot.org/uniprot/Q9Z2C5

The antiserum was produced against synthesized peptide derived from human Myotubularin. AA range:241-290

Myotubularin Polyclonal Antibody detects endogenous levels of Myotubularin protein.

WB 1:500-2000;IHC-p 1:50-300

70kD

-20°C/1 year

Polyclonal, Rabbit,IgG

This gene encodes a dual-specificity phosphatase that acts on both phosphotyrosine and phosphoserine. It is required for muscle cell differentiation and mutations in this gene have been identified as being responsible for X-linked myotubular myopathy. [provided by RefSeq, Jul 2008],

Epithelium,Platelet,Testis,

Cytoplasm . Cell membrane; Peripheral membrane protein . Cell projection, filopodium . Cell projection, ruffle . Late endosome . Cytoplasm, myofibril, sarcomere . Localizes as a dense cytoplasmic network (PubMed:11001925). Also localizes to the plasma membrane, including plasma membrane extensions such as filopodia and ruffles (PubMed:12118066). Predominantly located in the cytoplasm following interaction with MTMR12 (PubMed:12847286). Recruited to the late endosome following EGF stimulation (PubMed:14722070). In skeletal muscles, co-localizes with MTMR12 in the sarcomere (By similarity). .

catalytic activity:Protein tyrosine phosphate + H(2)O = protein tyrosine + phosphate.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:Defects in MTM1 are the cause of X-linked centronuclear myopathy X-linked (XCNM) [MIM:310400]; also known as X-linked myotubular myopathy (XLMTM) or myotubular myopathy type 1 (MTM1). Centronuclear myopathies are congenital muscle disorders characterized by progressive muscular weakness and wasting involving mainly limb girdle, trunk, and neck muscles. It may also affect distal muscles. Weakness may be present during childhood or adolescence or may not become evident until the third decade of life. Ptosis is a frequent clinical feature. The most prominent histopathologic features include high frequency of centrally located nuclei in muscle fibers not secondary to regeneration, radial arrangement of sarcoplasmic strands around the central nuclei, and predominance and hypotrophy of type 1 fibers.,function:Dual-specificity phosphatase that acts on both phosphotyrosine and phosphoserine. Could be involved in a signal transduction pathway necessary for late myogenesis, although its ubiquitous expression suggests a wider function.,similarity:Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.,similarity:Contains 1 GRAM domain.,similarity:Contains 1 myotubularin phosphatase domain.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.