Metabolism Antibodies 2
Anti-NEU1 Antibody (CAB6299)
- SKU:
- CAB6299
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
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 |