Monoclonal Anti-MTM1 antibody produced in mouse
SIGMA/WH0004534M1 - clone 1C10, purified immunoglobulin, buffered aqueous solution
Synonym: Anti-CNM; Anti-MTMX; Anti-XLMTM; Anti-myotubularin 1
Product Type: Chemical
Immunohistochemistry Immunoperoxidase of monoclonal antibody to MTM1 on formalin-fixed paraffin-embedded human pancreas. [antibody concentration 3 μg/mL]
Enhanced Validation-RNAi Western blot analysis of MTM1 over-expressed 293 cell line, cotransfected with MTM1 Validated Chimera RNAi. Blot probed with MTM1 monoclonal antibody, clone 1C10. GAPDH (36.1 kDa) used as specificity and loading control.
Western Blotting Western Blot analysis of MTM1 expression in transfected 293T cell line by MTM1 monoclonal antibody, clone 1C10. Lanes Lane 1: MTM1 transfected lysate (69.9 kDa). Lane 2: Non-transfected lysate.
Western Blotting QC Western Blot detection against Immunogen (36.63 kDa).
ELISA Detection limit for recombinant GST tagged MTM1 is approximately 1 ng/mL as a capture antibody.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | 1C10, monoclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| GenBank® accession no. | BC030779   |
| isotype | IgG2aκ |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable |
| indirect ELISA: suitable | |
| western blot: 1-5 μg/mL | |
| UniProt accession no. | Q13496 |
| Biochem/physiol Actions: | The MTM1 (myotubularin 1) targets two phosphoinositides (PIs), PtdIns3P and PtdInsP2, that participate in the endosomal–lysosomal pathway. Myotubularin is required for muscle cell differentiation and controls the mitochondrial morphology in muscular fibers. Mutations in the MTM1 gene result in X-linked myotubular myopathy (XLMTM), characterized by neonatal hypotonia and respiratory failure. |
| Disclaimer: | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
| General description: | The MTM1 (myotubularin 1) gene encodes myotubularin, a phosphatase that consists of 15 exons. It is located on human chromosome Xq28. |
| General description: | 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) |
| Immunogen: | MTM1 (AAH30779, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence MASASTSKYNSHSLENESIKRTSRD |
| Legal Information: | GenBank is a registered trademark of United States Department of Health and Human Services |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |