Anti-GALNS Antibody (CAB5461)
- SKU:
- CAB5461
- 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-GALNS Antibody |
| 抗体コード: | CAB5461 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 353-522 of human GALNS (NP_000503.1). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | MCF7, HT-29, 293T, Mouse kidney, Mouse brain, Mouse intestine |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 353-522 of human GALNS (NP_000503.1). |
| 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: | AGLT PPSD RAID GLNL LPTL LQGR LMDR PIFY YRGD TLMA ATLG QHKA HFWT WTNS WENF RQGI DFCP GQNV SGVT THNL EDHT KLPL IFHL GRDP GERF PLSF ASAE YQEA LSRI TSVV QQHQ EALV PAQP QLNV CNWA VMNW APPG CEKL GKCL TPPE SIPK KCLW SH |
| 遺伝子ID: | 2588 |
| Uniprot: | P34059 |
| セルラーロケーション: | Lysosome |
| 計算された分子量: | 58kDa |
| 観察された分子量: | 55kDa |
| 同義語: | GALNS, GALNAC6S, GAS, GalN6S, MPS4A |
| バックグラウンド: | This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder. |
| UniProt Protein Function: | GALNS: Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A); also known as Morquio A syndrome. MPS4A is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. Belongs to the sulfatase family. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.1.6.4 Chromosomal Location of Human Ortholog: 16q24.3 Cellular Component: lysosomal lumen Molecular Function:N-acetylgalactosamine-4-sulfatase activity; N-acetylgalactosamine-6-sulfatase activity; sulfuric ester hydrolase activity; metal ion binding Biological Process: keratan sulfate metabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process Disease: Mucopolysaccharidosis, Type Iva |
| NCBI Summary: | This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P34059 |
| NCBI GenInfo Identifier: | 462148 |
| NCBI Gene ID: | 2588 |
| NCBI Accession: | P34059.1 |
| UniProt Secondary Accession: | P34059,Q86VK3, |
| UniProt Related Accession: | P34059 |
| Molecular Weight: | 522 |
| NCBI Full Name: | N-acetylgalactosamine-6-sulfatase |
| NCBI Synonym Full Names: | galactosamine (N-acetyl)-6-sulfatase |
| NCBI Official Symbol: | GALNS |
| NCBI Official Synonym Symbols: | GAS; MPS4A; GalN6S; GALNAC6S |
| NCBI Protein Information: | N-acetylgalactosamine-6-sulfatase; chondroitinase; galNAc6S sulfatase; chondroitinsulfatase; galactose-6-sulfate sulfatase; N-acetylgalactosamine-6-sulfate sulfatase; galactosamine (N-acetyl)-6-sulfate sulfatase |
| UniProt Protein Name: | N-acetylgalactosamine-6-sulfatase |
| UniProt Synonym Protein Names: | Chondroitinsulfatase; Chondroitinase; Galactose-6-sulfate sulfatase; GalN6S; N-acetylgalactosamine-6-sulfate sulfatase; GalNAc6S sulfatase |
| Protein Family: | N-acetylgalactosamine-6-sulfatase |
| UniProt Gene Name: | GALNS |
| UniProt Entry Name: | GALNS_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GALNS antibody (CAB5461) 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: 15s. |
 | Immunofluorescence analysis of C6 cells using GALNS Rabbit pAb (CAB5461) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining. |
 | Immunofluorescence analysis of L929 cells using GALNS Rabbit pAb (CAB5461) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining. |
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