This antibody is purified through a protein A column, followed by peptide affinity purification.
Immunogen
This NEU1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 188-214 amino acids from the Central region of human NEU1.
NEU1
Reaktivität: Human
WB, IHC
Wirt: Kaninchen
Polyclonal
unconjugated
Applikationshinweise
WB: 1:1000
Beschränkungen
Nur für Forschungszwecke einsetzbar
Format
Liquid
Buffer
Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Lagerung
4 °C,-20 °C
Haltbarkeit
6 months
Caciotti, Di Rocco, Filocamo, Grossi, Traverso, dAzzo, Cavicchi, Messeri, Guerrini, Zammarchi, Donati, Morrone: "Type II sialidosis: review of the clinical spectrum and identification of a new splicing defect with chitotriosidase assessment in two patients." in: Journal of neurology, Vol. 256, Issue 11, pp. 1911-5, (2009) (PubMed).
Lai, Chen, Wu Chou, Chang, Kao, Huang, Weng, Chen, Hwu, Lu: "A longitudinal study of Taiwanese sialidosis type 1: an insight into the concept of cherry-red spot myoclonus syndrome." in: European journal of neurology : the official journal of the European Federation of Neurological Societies, Vol. 16, Issue 8, pp. 912-9, (2009) (PubMed).
Bonten, Campos, Zaitsev, Nourse, Waddell, Lewis, Taylor, dAzzo: "Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization." in: The Journal of biological chemistry, Vol. 284, Issue 41, pp. 28430-41, (2009) (PubMed).
The protein encoded by this gene is a lysosomal enzyme that cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids. In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter is also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red spot-myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity.