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- gelatins
- jlcR15543
- 国内
- 2025年07月02日
- WB,ELISA等
- 人/动物/植物
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- 详细信息
- 文献和实验
- 技术资料
- 供应商：
江西江蓝纯生物试剂有限公司
- 库存：
83
- 克隆性：
单克隆
- 保质期：
1年
- 抗体英文名：
Iduronate 2 sulfatase
- 抗体名：
艾杜糖-2-硫酸酯酶抗体
- 适应物种：
人/动物/植物
- 应用范围：
WB,ELISA等
- 浓度：
1mg/ml
- 保存条件：
-20 °
- 规格：
100ul/200ul
| 规格： | 100ul | 产品价格： | ¥1580.0 |
|---|---|---|---|
| 规格： | 200ul | 产品价格： | ¥2480.0 |
产品货号 : jlcR15543
英文名称 : Iduronate 2 sulfatase
中文名称 : 艾杜糖-2-硫酸酯酶抗体
别 名 : Alpha L iduronate sulfate sulfatase; Alpha-L-iduronate sulfate sulfatase; AW214631; Ids; IDS_HUMAN; Iduronate 2 sulfatase 14 kDa chain; Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate sulfatase; Idursulfase; MPS2; RP23-29M4.1; SIDS.
研究领域 : 细胞生物 免疫学 细胞类型标志物
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Rat, Dog, Cow, 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 : 47kDa
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 Iduronate 2 sulfatase:101-200/550
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 : The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013]
Function:
Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
Subunit:
Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a 42 kDa and a 14 kDa polypeptides.
Subcellular Location:
Lysosome.
Tissue Specificity:
Liver, kidney, lung, and placenta.
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:
Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the sulfatase family.
SWISS:
P22304
Gene ID:
3423
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品图片
英文名称 : Iduronate 2 sulfatase
中文名称 : 艾杜糖-2-硫酸酯酶抗体
别 名 : Alpha L iduronate sulfate sulfatase; Alpha-L-iduronate sulfate sulfatase; AW214631; Ids; IDS_HUMAN; Iduronate 2 sulfatase 14 kDa chain; Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate sulfatase; Idursulfase; MPS2; RP23-29M4.1; SIDS.
研究领域 : 细胞生物 免疫学 细胞类型标志物
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Rat, Dog, Cow, 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 : 47kDa
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 Iduronate 2 sulfatase:101-200/550
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 : The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013]
Function:
Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
Subunit:
Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a 42 kDa and a 14 kDa polypeptides.
Subcellular Location:
Lysosome.
Tissue Specificity:
Liver, kidney, lung, and placenta.
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:
Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the sulfatase family.
SWISS:
P22304
Gene ID:
3423
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品图片
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文献和实验相关实验
。但是，最近，在（ 1）的酶中有作用于天然硫酸（脑苷）脂（脂类的硫酸酯）和糖核苷（ UDP－ N-乙酰半乳糖胺 -4-硫酸）的酶，另在（ 3）的酶中发现作用于硫酸软骨素 B的艾杜糖醛酸的硫酸基的酶。此外还知道，有好几种遗传性疾病是由于缺少这些酶的缘故，并越来越认识到在生物体内硫酸酯代谢的重要性。
症（ Hunter syndrome）和常染色体性劣性的哈特综合症（ Hurter syndrome）都是硫酸皮肤素分解系统异常所致，前者缺少水解结合于 L-艾杜糖醛酸的硫酸基的硫酸酯酶，后者缺少从非还原末端分解 L-艾杜糖醛酸的α－ L-艾杜糖醛酸酶〔 EC3． 2． 1． 76）。每种病都以面貌和骨骼的异常，脾脏肿大，智能障碍为基本所见，尿中有异常多的皮肤素硫酸和乙酰肝素硫酸。
结缔组织生化(Biochemistryof Connective Tissue)
，糖链合成后再进一步修饰。由差向异构酶催化将葡萄糖醛酸转变为艾杜糖醛酸，而硫酸基则是由活性硫酸根(PAPS)提供，由硫酸转移酶 图13－3 糖胺多糖链与多肽链的连接 催化加到新合成的多糖链的氨基或羟基上。(图13-4为硫酸软骨素A合成过程示意图)，当Vit A缺乏时硫酸转移酶活性下降，糖胺多糖合成受限，可引起组织生长、发育的障碍。 图13－4 硫酸软骨素A的合成示意 四、蛋白多糖的分解代谢 结缔组织基质中的蛋白
艾杜糖-2-硫酸酯酶抗体
¥1580 - 2480
