GNE Antikörper (AbBy Fluor® 750)

Produktnummer ABIN5003526

This anti-GNE antibody (ABIN5003526) is a Rabbit Polyclonal antibody detecting GNE in WB, IF (p). Suitable for Human, Mouse, Rat.

Kurzübersicht für GNE Antikörper (AbBy Fluor® 750) (ABIN5003526)

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® 750

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

Produktdetails anti-GNE Antikörper (AbBy Fluor® 750)

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

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