Anti-GNS Antibody (CAB7489)
- SKU:
- CAB7489
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-GNS Antibody |
| 抗体コード: | CAB7489 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 293-552 of human GNS (NP_002067.1). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | BT-474, NCI-H460, 293T, SKOV3, HeLa, Mouse liver, Mouse kidney, Mouse spleen, Rat brain |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 293-552 of human GNS (NP_002067.1). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | WQTL LSVD DLVE KLVK RLEF TGEL NNTY IFYT SDNG YHTG QFSL PIDK RQLY EFDI KVPL LVRG PGIK PNQT SKML VANI DLGP TILD IAGY DLNK TQMD GMSL LPIL RGAS NLTW RSDV LVEY QGEG RNVT DPTC PSLS PGVS QCFP DCVC EDAY NNTY ACVR TMSA LWNL QYCE FDDQ EVFV EVYN LTAD PDQI TNIA KTID PELL GKMN YRLM MLQS CSGP TCRT PGVF DPGY RFDP RLMF SNRG SVRT RRFS KHLL |
| 遺伝子ID: | 2799 |
| Uniprot: | P15586 |
| セルラーロケーション: | Lysosome |
| 計算された分子量: | 59kDa/62kDa |
| 観察された分子量: | 62kDa |
| 同義語: | GNS, G6S |
| バックグラウンド: | The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome. |
| UniProt Protein Function: | GNS: Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D); also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Belongs to the sulfatase family. |
| UniProt Protein Details: | Protein type:EC 3.1.6.14; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation Chromosomal Location of Human Ortholog: 12q14 Cellular Component: lysosomal lumen Molecular Function:protein binding; N-acetylglucosamine-6-sulfatase activity; sulfuric ester hydrolase activity; metal ion binding Biological Process: keratan sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process Disease: Mucopolysaccharidosis, Type Iiid |
| NCBI Summary: | The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P15586 |
| NCBI GenInfo Identifier: | 232126 |
| NCBI Gene ID: | 2799 |
| NCBI Accession: | P15586.3 |
| UniProt Related Accession: | P15586 |
| Molecular Weight: | |
| NCBI Full Name: | N-acetylglucosamine-6-sulfatase |
| NCBI Synonym Full Names: | glucosamine (N-acetyl)-6-sulfatase |
| NCBI Official Symbol: | GNS |
| NCBI Official Synonym Symbols: | G6S |
| NCBI Protein Information: | N-acetylglucosamine-6-sulfatase |
| UniProt Protein Name: | N-acetylglucosamine-6-sulfatase |
| UniProt Synonym Protein Names: | Glucosamine-6-sulfatase; G6S |
| Protein Family: | Protein |
| UniProt Gene Name: | GNS |
| UniProt Entry Name: | GNS_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GNS antibody (CAB7489) 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. |
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