Recombinant Human GNS Protein

重组人GNS蛋白 | 重组人葡萄糖胺(N-乙酰基)-6-硫酸酯酶蛋白

EC 3.1.6 | EC 3.1.6.14 | G6Sglucosamine-6-sulfatase | glucosamine (N-acetyl)-6-sulfatase | Glucosamine6Sulfatase | Glucosamine-6-Sulfatase | GNS | MGC21274 | N-acetylglucosamine-6-sulfatase

rp184039-10μg

10μg

无动物源

≥95%(SDS-PAGE)

A member of the sulfatase family, GNS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and keratan sulfate. It hydrolyzes the 6-sulfate group of the N-acetyl-D-glucosamine 6-sulfate units of the GAG. GNS deficiency results in mucopolysaccharidosis type IIID (MPS IIID or Sanfilippo D Syndrome), an inborn error leading to lysosomal accumulation of heparan sulfate. MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. The deduced amino acid sequence of human GNS consists of a signal peptide (residues 1-36) and a mature chain (residues 37-552) that may be further processed into N-terminal and C-terminal fragments. rhGNS corresponds to the single chain and has sulfatase activity described in Activity Assay Protocol. Post-translational: The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species. The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.

Testing in progress

重组表达

59.6 kDa

C-10His

80.6 kDa, under reducing conditions; 80.6 kDa, under non-reducing conditions.

HEK293 Accession #: P15586 | HEK293

<1.0 EU/μg

人(Human)

1-552 aa

MRLLPLAPGRLRRGSPRHLPSCSPALLLLVLGGCLGVFGVAAGTRRPNVVLLLTDDQDEVLGGMTPLKKTKALIGEMGMTFSSAYVPSALCCPSRASILTGKYPHNHHVVNNTLEGNCSSKSWQKIQEPNTFPAILRSMCGYQTFFAGKYLNEYGAPDAGGLEHVPLGWSYWYALEKNSKYYNYTLSINGKARKHGENYSVDYLTDVLANVSLDFLDYKSNFEPFFMMIATPAPHSPWTAAPQYQKAFQNVFAPRNKNFNIHGTNKHWLIRQAKTPMTNSSIQFLDNAFRKRWQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSLPIDKRQLYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDLNKTQMDGMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDCVCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPDQITNIAKTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMFSNRGSVRTRRFSKHLLHHHHHHHHHH

Yes

Yes