产品中心
当前位置：首页>产品中心Anti-GALNS
货号： bs-13269R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-13269R
- 英文名称
- GALNS
- 中文名称
- 硫酸软骨素裂解酶抗体
- 别 名
- Chondroitinase; Chondroitinsulfatase; FLJ00319; FLJ17434; FLJ42844; FLJ98217; Galactosamine (N acetyl) 6 sulfate sulfatase; Galactose 6 sulfate sulfatase; GALNAC6S; GalNAc6S sulfatase; GAS; mFLJ00319; MPS4A; N acetylgalactosamine 6 sulfatase; N acetylgalactosamine 6 sulfate sulfatase; GALNS_HUMAN.
- 规格价格
- 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 研究领域
- 细胞生物 信号转导 泛素
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
- 产品应用
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 55kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human GALNS:1-100/522
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- PubMed
- PubMed
- 产品介绍
- background:
Chondroitinase is a 522 amino acid protein that localizes to the lysosome and functions as an exohydrolase that is essential for the degradation of glycosaminoglycans, keratan sulfate and chondroitin 6-sulfate. Using calcium as a cofactor, Chondroitinase, which exists as a disulfide linked oligomer, catalyzes the hydrolysis of the 6-sulfate group on target substrates. Defects in the gene encoding Chondroitinase are the cause of mucopolysaccharidosis type 4A (MPS4A), an autosomal recessive lysosomal storage disease that is characterized by the intracellular accumulation of keratan sulfate and chondroitin-6-sulfate and is associated with dental anomalies, short stature and, in some cases, death in the second or third decade of life.
Function:
GALNS (N-acetylgalactosamine-6-sulfatase) is a lysosomal exohydrolase which catalyzes the hydrolysis of the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate and of the D-galactose 6-sulfate units of keratan sulfate. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.
Subunit:
Oligomer of disulfide linked 40- and 15 kDa polypeptides.
Subcellular Location:
Lysosome.
Post-translational modifications:
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 (By similarity).
DISEASE:
Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A) [MIM:253000]; 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.
Similarity:
Belongs to the sulfatase family.
SWISS:
P34059
Gene ID:
2588
Database links:Entrez Gene: 2588 Human
Entrez Gene: 50917 Mouse
Entrez Gene: 292073 Rat
Omim: 612222 Human
SwissProt: P34059 Human
SwissProt: Q571E4 Mouse
SwissProt: Q32KJ6 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.