protein

NEu1, 48-415aa, Human, His tag, E Coli

MBS204313

0.1 mg

375 USD

Recombinant Protein

NEu1, 48-415aa, Human, His tag, E Coli

NEu1

Sialidase 1 (lysosomal sialidase); NANH; NEu; SIAL1

sialidase-1; Sialidase-1; sialidase-1; G9 sialidase; N-acetyl-alpha-neuraminidase 1; acetylneuraminyl hydrolase; exo-alpha-sialidase; lysosomal sialidase; sialidase 1 (lysosomal sialidase); Acetylneuraminyl hydrolase; G9 sialidase; Lysosomal sialidase; N-acetyl-alpha-neuraminidase 1

NEu1

NEU1; NEU1; NEU; NANH; SIAL1; NANH

NEUR1_HUMAN

415

GSSHHHHHH SSGLVPRGSH MGSHMENDFG LVQPLVTMEQ LLWVSGRQIG SVDTFRIPLI TATPRGTLLA FAEARKMSSS DEGAKFIALR RSMDQGSTWS PTAFIVNDGD VPDGLNLGAV VSDVETGVVF LFYSLCAHKA GCQVASTMLV WSKDDGVSWS TPRNLSLDIG TEVFAPGPGS GIQKQREPRK GRLIVCGHGT LERDGVFCLL SDDHGASWRY GSGVSGIPYG QPKQENDFNP DECQPYELPD GSVVINARNQ NNYHCHCRIV LRSYDACDTL RPRDVTFDPE LVDPVVAAGA VVTSSGIVFF SNPAHPEFRV NLTLRWSFSN GTSWRKETVQ LWPGPSGYSS LATLEGSMDG EEQAPQLYVL YEKGRNHYTE SISVAKISVY GTL

> 85% by SDS - PAGE

Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 0.15M NaCl, 10% glycerol,1mM DTT

0.25 mg/ml (determined by Bradford assay)

Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.

SDS-PAGE

Antigen Species: Human. Tag: His-tag

Expression System: E Coli

Metabolism

NEu1 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. Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red -myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity. Recombinant human NEu1 protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Abdulkhalek,S., et al. (2011) J. Biol. Chem. 286 (42), 36532-36549

4557791

NP_000425

NM_000434.3

42.9 kDa (393aa), confirmed by MALDI-TOF

Asparagine N-linked Glycosylation Pathway 161013!!Biosynthesis Of The N-glycan Precursor (dolichol Lipid-linked Oligosaccharide, LLO) And Transfer To A Nascent Protein Pathway 161014!!Defective ALG1 Causes ALG1-CDG (CDG-1k) Pathway 1127605!!Defective ALG11 Causes ALG11-CDG (CDG-1p) Pathway 1127608!!Defective ALG12 Causes ALG12-CDG (CDG-1g) Pathway 1127601!!Defective ALG14 Causes Congenital Myasthenic Syndrome (ALG14-CMS) Pathway 1127613!!Defective ALG2 Causes ALG2-CDG (CDG-1i) Pathway 1127603!!Defective ALG3 Causes ALG3-CDG (CDG-1d) Pathway 1127599!!Defective ALG6 Causes ALG6-CDG (CDG-1c) Pathway 1127597!!Defective ALG8 Causes ALG8-CDG (CDG-1h) Pathway 1127602

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]

0.1 mg

375 USD