Anti-NEU1 항체 (CAB6299)

Antibody Name:	Anti-NEU1 Antibody
Antibody SKU:	CAB6299
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 156-415 of human NEU1 (NP_000425.1).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	BxPC-3, Mouse kidney, Mouse pancreas, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 156-415 of human NEU1 (NP_000425.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	SLCA HKAG CQVA STML VWSK DDGV SWST PRNL SLDI GTEV FAPG PGSG IQKQ REPR KGRL IVCG HGTL ERDG VFCL LSDD HGAS WRYG SGVS GIPY GQPK QEND FNPD ECQP YELP DGSV VINA RNQN NYHC HCRI VLRS YDAC DTLR PRDV TFDP ELVD PVVA AGAV VTSS GIVF FSNP AHPE FRVN LTLR WSFS NGTS WRKE TVQL WPGP SGYS SLAT LEGS MDGE EQAP QLYV LYEK GRNH YTES ISVA KISV YGTL
Gene ID:	4758
Uniprot:	Q99519
Cellular Location:	Cell membrane, Cytoplasmic vesicle, Lumenal side, Lysosome lumen, Lysosome membrane, Peripheral membrane protein
Calculated MW:	45kDa
Observed MW:	45KDa

Synonyms:

NEU1, NANH, NEU, SIAL1

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.

UniProt Protein Function:	NEU1: Catalyzes the removal of sialic acid (N-acetylneuramic acid) moities from glycoproteins and glycolipids. To be active, it is strictly dependent on its presence in the multienzyme complex. Appears to have a preference for alpha 2-3 and alpha 2-6 sialyl linkage. Defects in NEU1 are the cause of sialidosis (SIALIDOSIS). It is a lysosomal storage disease occurring as two types with various manifestations. Type 1 sialidosis (cherry red spot-myoclonus syndrome or normosomatic type) is late-onset and it is characterized by the formation of cherry red macular spots in childhood, progressive debilitating myoclonus, insiduous visual loss and rarely ataxia. The diagnosis can be confirmed by the screening of the urine for sialyloligosaccharides. Type 2 sialidosis (also known as dysmorphic type) occurs as several variants of increasing severity with earlier age of onset. It is characterized by the presence of abnormal somatic features including coarse facies and dysostosis multiplex, vertebral deformities, mental retardation, cherry-red spot/myoclonus, sialuria, cytoplasmic vacuolation of peripheral lymphocytes, bone marrow cells and conjunctival epithelial cells. Belongs to the glycosyl hydrolase 33 family.
UniProt Protein Details:	Protein type:Hydrolase; EC 3.2.1.18; Lipid Metabolism - sphingolipid; Motility/polarity/chemotaxis; Glycan Metabolism - other glycan degradation Chromosomal Location of Human Ortholog: 6p21.3 Cellular Component: lysosomal lumen; intracellular membrane-bound organelle; lysosome; lysosomal membrane; cytoplasmic membrane-bound vesicle; plasma membrane; cell junction Molecular Function:exo-alpha-sialidase activity Biological Process: oligosaccharide catabolic process; cellular protein metabolic process; sphingolipid metabolic process; dolichol-linked oligosaccharide biosynthetic process; glycosphingolipid metabolic process; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification; lipid catabolic process Disease: Neuraminidase Deficiency
NCBI Summary:	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. [provided by RefSeq, Jul 2008]
UniProt Code:	Q99519
NCBI GenInfo Identifier:	17368612
NCBI Gene ID:	4758
NCBI Accession:	Q99519.1
UniProt Related Accession:	Q99519
Molecular Weight:	415
NCBI Full Name:	Sialidase-1
NCBI Synonym Full Names:	sialidase 1 (lysosomal sialidase)
NCBI Official Symbol:	NEU1
NCBI Official Synonym Symbols:	NEU; NANH; SIAL1
NCBI Protein Information:	sialidase-1; G9 sialidase; exo-alpha-sialidase; lysosomal sialidase; acetylneuraminyl hydrolase; N-acetyl-alpha-neuraminidase 1
UniProt Protein Name:	Sialidase-1
UniProt Synonym Protein Names:	Acetylneuraminyl hydrolase; G9 sialidase; Lysosomal sialidase; N-acetyl-alpha-neuraminidase 1
Protein Family:	Sialidase
UniProt Gene Name:	NEU1
UniProt Entry Name:	NEUR1_HUMAN

	Western blot analysis of extracts of various cell lines, using NEU1 antibody (CAB6299) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunohistochemistry of paraffin-embedded mouse kidney using NEU1 Rabbit pAb (CAB6299) at dilution of 1:100 (40x lens).

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)