人Acid sphingomyelinase抗体Pair - BSA and Azide free (SMPD1) (ab253462)
Key features and details
- Unconjugated capture and detector antibodies
- Adaptable to any antibody pair-based assay format
- Antibody concentration ~ 1 mg/ml
- BSA and azide free buffer - ready for conjugation
- Reacts with: Human
概述
-
产品名称
人Acid sphingomyelinase抗体Pair - BSA and Azide free (SMPD1)
参阅全部 Acid sphingomyelinase 试剂盒 -
检测类型
ELISA set -
范围
125 pg/ml - 8000 pg/ml -
种属反应性
与反应: Human -
产品概述
Both capture and detector antibodies are recombinant rabbit monoclonal antibodies delivering consistent, specific, and sensitive results.
The Antibody Pair can be used to quantify Human Acid sphingomyelinase. BSA and Azide free antibody pairs include unconjugated capture and detector antibodies suitable for sandwich ELISAs. The antibodies are provided at an approximate concentration of 1 mg/ml as measured by the protein A280 method. The recommended antibody orientation is based on internal optimization for ELISA-based assays. Antibody orientation is assay dependent and needs to be optimized for each assay type.
For additional information on the performance of the antibody pair, see the equivalent SimpleStep ELISA® Kit (ab277075), which uses the same antibodies. However, due to differences in their formulation, this antibody pair cannot be used with the consumables provided with our SimpleStep ELISA Kits. Please note that the range provided for the pairs is only an estimation based on the performance of the related product using the same antibody pair. Performance of the antibody pair will depend on the specific characteristics of your assay. We guarantee the product works in sandwich ELISA, but we do not guarantee the sensitivity or dynamic range of the antibody pair in your assay.
Download SDS here.
-
经测试应用
适用于: Sandwich ELISAmore details -
平台
Reagents
性能
-
存放说明
Store at +4°C. Please refer to protocols. -
无载体
是 -
组件 10 x 96 tests Human Acid sphingomyelinase Capture Antibody (unconjugated) 1 x 100µg Human Acid sphingomyelinase Detector Antibody (unconjugated) 1 x 100µg -
研究领域
-
功能
Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity. -
疾病相关
Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) [MIM:257200]; also known as Niemann-Pick disease classical infantile form. It is an early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, mental retardation, digestive disorders, failure to thrive, major hepatosplenomegaly, and severe neurologic symptoms. The severe neurological disorders and pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B.
Defects in SMPD1 are the cause of Niemann-Pick disease type B (NPDB) [MIM:607616]; also known as Niemann-Pick disease visceral form. It is a late-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Clinical signs involve only visceral organs. The most constant sign is hepatosplenomegaly which can be associated with pulmonary symptoms. Patients remain free of neurologic manifestations. However, a phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B. In Niemann-Pick disease type B, onset of the first symptoms occurs in early childhood and patients can survive into adulthood. -
序列相似性
Belongs to the acid sphingomyelinase family.
Contains 1 saposin B-type domain. -
细胞定位
Lysosome. - Information by UniProt
-
别名
- Acid sphingomyelinase
- ASM
- ASM_HUMAN
see all -
数据库链接
- Entrez Gene: 6609 Human
- Omim: 607608 Human
- SwissProt: P17405 Human
- Unigene: 498173 Human
相关产品
-
Alternative Versions
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab253462于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
---|---|---|
Sandwich ELISA |
Use at an assay dependent concentration.
|
说明 |
---|
Sandwich ELISA
Use at an assay dependent concentration. |
图片
-
Representative standard curve from corresponding SimpleStep ELISA® Kit (ab277075), which uses the same antibody pair. For additional information on the performance of pair and kit, refer to the corresponding kit datasheet. Due to differences in the formulation and format of the antibodies in this pair, they cannot be used as substitutes for the antibody components in our SimpleStep ELISA® Kits.
-
To learn more about the advantages of recombinant antibodies see here.
实验方案
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
数据表及文件
-
Datasheet download
文献 (0)
ab253462 尚未被引用在任何文献中。