MTM1 Antikörper (AA 225-275) (AbBy Fluor® 647)

Produktnummer ABIN1401288

Produktdetails anti-MTM1 Antikörper (AbBy Fluor® 647)

Target: MTM1 (Myotubularin 1 (MTM1))

Bindungsspezifität: AA 225-275

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser MTM1 Antikörper ist konjugiert mit AbBy Fluor® 647

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

Anwendungsinformationen

Applikationshinweise: FCM 1:20-100
IF(IHC-P) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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

Details zu MTM1

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:

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