GNE Antikörper (AbBy Fluor® 594)

Produktnummer ABIN2813765

Produktdetails anti-GNE Antikörper (AbBy Fluor® 594)

Target: GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GNE Antikörper ist konjugiert mit AbBy Fluor® 594

Applikation: Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Kreuzreaktivität: Human, Maus, Ratte

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GLCNE

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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 GNE

Target: GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))

Andere Bezeichnung: GLCNE (GNE Produkte)

Synonyme: DMRV antikoerper, GLCNE antikoerper, IBM2 antikoerper, UAE1 antikoerper, zgc:77657 antikoerper, GNE antikoerper, MGC145505 antikoerper, NM antikoerper, Uae1 antikoerper, 2310066H07Rik antikoerper, glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase antikoerper, GNE antikoerper, gne antikoerper, Gne antikoerper

Hintergrund:

Synonyms: IBM2, Uae1, Bunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase, DMRV, ManAc kinase, N acylmannosamine kinase, NM, RP23-209M8.6, UDP GlcNAc 2 epimerase, UDP GlcNAc 2 epimerase/ManAc kinase, Uridine diphosphate N acetylglucosamine 2 epimerase, GLCNE_HUMAN.

Background: The bifunctional enzyme UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/Mnk), or GLCNE, regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. GLCNE is required for normal sialylation in hematopoietic cells. Sialylation is implicated in cell adhesion, signal transduction, tumorigenicity and metastatic behavior of malignant cells. It is upregulated after PKC-dependent phosphorylation and is most abundantly expressed in liver and placenta. It is also expressed, to a lesser extent, in heart, brain, lung, kidney, skeletal muscle and pancreas. Defects in GLCNE are the cause of sialuria, inclusion body myopathy 2 (IBM2) and Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles (DMRV). Sialuria is an autosomal dominant disorder caused by a lack of feedback inhibition of GLCNE by CMP-NeuAc, resulting in overproduction of NeuAc. It is characterized by an accumulation of free sialic acid in the cytoplasm and large quantities of neuraminic acid in the urine. Both IBM2 and NM/DMRV are autosomal recessive neuromuscular disorders characterized by adult onset, distal and proximal muscle weakness (especially in the legs) and a typical muscle pathology including filamentous inclusions and rimmed vacuoles.

Gen-ID: 10020