GNE anticorps (AbBy Fluor® 750)

N° du produit ABIN5003526

Détail du produit anti-GNE anticorps (AbBy Fluor® 750)

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GNE est conjugé à/à la AbBy Fluor® 750

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

Réactivité croisée: Humain, Souris, Rat

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human GLCNE

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur GNE

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

Autre désignation: GLCNE (GNE Produits)

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

Sujet:

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.

ID gène: 10020