MTM1 Antikörper (AA 225-275) (AbBy Fluor® 594)
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- Target Alle MTM1 Antikörper anzeigen
- MTM1 (Myotubularin 1 (MTM1))
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Bindungsspezifität
- AA 225-275
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Reaktivität
- Human, Maus, Ratte
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Wirt
- Kaninchen
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Klonalität
- Polyklonal
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Konjugat
- Dieser MTM1 Antikörper ist konjugiert mit AbBy Fluor® 594
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Applikation
- Western Blotting (WB), Flow Cytometry (FACS), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Kreuzreaktivität
- Human, Maus, Ratte
- Aufreinigung
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human MTM1/Myotubularin
- Isotyp
- IgG
- Top Product
- Discover our top product MTM1 Primärantikörper
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- Applikationshinweise
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FCM 1:20-100
IF(IHC-P) 1:50-200 - Beschränkungen
- Nur für Forschungszwecke einsetzbar
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- Format
- Liquid
- Konzentration
- 1 μg/μL
- Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- Konservierungsmittel
- ProClin
- Vorsichtsmaßnahmen
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Lagerung
- -20 °C
- Informationen zur Lagerung
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Haltbarkeit
- 12 months
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- Target
- MTM1 (Myotubularin 1 (MTM1))
- Andere Bezeichnung
- MTM1 (MTM1 Produkte)
- Synonyme
- CNM antikoerper, MTMX antikoerper, XLMTM antikoerper, wu:fb19c01 antikoerper, zgc:123266 antikoerper, myotubularin 1 antikoerper, myotubularin 1 S homeolog antikoerper, MTM1 antikoerper, mtm1 antikoerper, mtm1.S antikoerper, Mtm1 antikoerper
- Hintergrund
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Synonyms: CG2, CNM, KIAA4176, mKIAA4176, Mtm, Mtm1, MTM1_HUMAN, MTMX, Myotubular myopathy 1, Myotubularin, XLMTM.
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.
- Gen-ID
- 4534
- UniProt
- Q13496
- Pathways
- Inositol Metabolic Process, Skeletal Muscle Fiber Development
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