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Western Blot (WB) ((0.3ug/ml) staining of Human Skeletal Muscle lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Goat MTM1 Polyclonal Antibody | anti-MTM1 antibody

Goat anti-MTM1 Antibody

Gene Names
MTM1; CNM; MTMX; XLMTM
Reactivity
Tested: Human, Mouse; Expected from sequence similarity: Human, Mouse, Dog, Cow
Applications
ELISA, Western Blot
Purity
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Synonyms
MTM1; Polyclonal Antibody; Goat anti-MTM1 Antibody; myotubular myopathy 1; CNM; MTMX; XLMTM; myotubularin; myotubularin 1; MTM1 antibody; myotubular myopathy 1 antibody; CNM antibody; MTMX antibody; XLMTM antibody; myotubularin antibody; myotubularin 1 antibody; anti-MTM1 antibody
Ordering
For Research Use Only!
Host
Goat
Reactivity
Tested: Human, Mouse; Expected from sequence similarity: Human, Mouse, Dog, Cow
Clonality
Polyclonal
Purity/Purification
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Form/Format
Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
Concentration
100ug specific antibody in 200ul (varies by lot)
Sequence
SSPSQMMPHVQTHF
Sequence Length
603
Applicable Applications for anti-MTM1 antibody
Peptide ELISA (EIA), Western Blot (WB)
Application Notes
Peptide ELISA: Antibody detection limit dilution 1: 8000.
Western Blot: Approx 70kDa band observed in lysates of Human Skeletal Muscle, Mouse Heart and Skeletal Muscle and of cell line HepG2 (calculated MW of 69.9kDa according to NP_000243.1and 69.6kDa according to Mouse NP_001157662.1). Recommended concentration: 1-3ug/ml.
Immunogen
Peptide with sequence C-SSPSQMMPHVQTHF, from the C Terminus of the protein sequence according to NP_000243.1.
Epitope
C Terminus
Preparation and Storage
Aliquot and store at -20 degree C. Minimize freezing and thawing.

Western Blot (WB)

((0.3ug/ml) staining of Human Skeletal Muscle lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Western Blot (WB) ((0.3ug/ml) staining of Human Skeletal Muscle lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Western Blot (WB)

((2ug/ml) staining of HepG2 cell lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Western Blot (WB) ((2ug/ml) staining of HepG2 cell lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Western Blot (WB)

((1ug/ml) staining of Mouse Heart (A) and Skeletal Muscle (B) lysates (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Western Blot (WB) ((1ug/ml) staining of Mouse Heart (A) and Skeletal Muscle (B) lysates (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
Molecular Weight
66,053 Da
NCBI Official Full Name
myotubularin
NCBI Official Synonym Full Names
myotubularin 1
NCBI Official Symbol
MTM1
NCBI Official Synonym Symbols
CNM; MTMX; XLMTM
NCBI Protein Information
myotubularin
UniProt Protein Name
Myotubularin
Protein Family
UniProt Gene Name
MTM1
UniProt Synonym Gene Names
CG2
UniProt Entry Name
MTM1_HUMAN

NCBI 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, Jul 2008]

Uniprot Description

MTM1: Lipid phosphatase which dephosphorylates phosphatidylinositol 3-monophosphate (PI3P) and phosphatidylinositol 3,5-bisphosphate (PI(3,5)P2). Has also been shown to dephosphorylate phosphotyrosine- and phosphoserine- containing peptides. Negatively regulates EGFR degradation through regulation of EGFR trafficking from the late endosome to the lysosome. Plays a role in vacuolar formation and morphology. Regulates desmin intermediate filament assembly and architecture. Plays a role in mitochondrial morphology and positioning. Required for skeletal muscle maintenance but not for myogenesis. Defects in MTM1 are the cause of centronuclear myopathy X-linked (CNMX). A congenital muscle disorder 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. Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.

Protein type: EC 3.1.3.95; Protein phosphatase, dual-specificity; EC 3.1.3.64; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: cytoplasm; cytosol; filopodium; I band; late endosome; plasma membrane; ruffle

Molecular Function: intermediate filament binding; phosphatidylinositol-3-phosphatase activity; phosphoinositide binding; phosphoprotein phosphatase activity; protein binding; protein tyrosine phosphatase activity

Biological Process: endosome to lysosome transport; intermediate filament organization; mitochondrion distribution; muscle maintenance; negative regulation of proteasomal ubiquitin-dependent protein catabolic process; negative regulation of protein kinase B signaling cascade; negative regulation of TOR signaling pathway; phosphatidylinositol biosynthetic process; phosphoinositide dephosphorylation; positive regulation of skeletal muscle growth; protein amino acid dephosphorylation; protein transport

Disease: Myopathy, Centronuclear, X-linked

Research Articles on MTM1

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Product Notes

The MTM1 mtm1 (Catalog #AAA420871) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Goat anti-MTM1 Antibody reacts with Tested: Human, Mouse; Expected from sequence similarity: Human, Mouse, Dog, Cow and may cross-react with other species as described in the data sheet. AAA Biotech's MTM1 can be used in a range of immunoassay formats including, but not limited to, Peptide ELISA (EIA), Western Blot (WB). Peptide ELISA: Antibody detection limit dilution 1: 8000. Western Blot: Approx 70kDa band observed in lysates of Human Skeletal Muscle, Mouse Heart and Skeletal Muscle and of cell line HepG2 (calculated MW of 69.9kDa according to NP_000243.1and 69.6kDa according to Mouse NP_001157662.1). Recommended concentration: 1-3ug/ml. Researchers should empirically determine the suitability of the MTM1 mtm1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: SSPSQMMPHV QTHF. It is sometimes possible for the material contained within the vial of "MTM1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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