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  • 产品名称：anti-NEU1antibody(Sialidase1(LysosomalSialidase))(AA188-214)

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简单介绍：
anti-NEU1antibody(Sialidase1(LysosomalSialidase))(AA188-214)
详情介绍：
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.
Clone RB42166
Predicted Reactivity Cow (Bovine)
Purification This antibody is purified through a protein A column, followed by peptide affinity purification. Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
Alternative Name NEU1 (NEU1 Antibody Abstract)
Background 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.

Synonyms: NEU1, NANH, Sialidase-1, Acetylneuraminyl hydrolase, G9 sialidase, Lysosomal sialidase, N-acetyl-alpha-neuraminidase 1
NCBI Accession NP_000425
UniProt Q99519
Application Notes WB: 1:1000
Restrictions For Research Use only
Format Liquid
Concentration 0.5 mg/mL
Buffer Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
Preservative Sodium azide
Precaution of Use WARNING: Reagents contain sodium azide. Sodium azide is very toxic if ingested or inhaled. Avoid contact with skin, eyes, or clothing. Wear eye or face protection when handling. If skin or eye contact occurs, wash with copious amounts of water. If ingested or inhaled, contact a physician immediately. Sodium azide yields toxic hydrazoic acid under acidic conditions. Dilute azide-containing compounds in running water before discarding to avoid accumulation of potentially explosive deposits in lead or copper plumbing.
Supplier Images
Western Blotting (WB) image for anti-Sialidase 1 (Lysosomal Sialidase) (NEU1) (AA 188-214), (Center) antibody (ABIN1881576) NEU1 Antibody (Center) (ABIN1881576) western blot analysis in HepG2 cell line lysates...
Product cited in: 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).