SMPD1 抗体 (AA 201-300) (Cy7)
Quick Overview for SMPD1 抗体 (AA 201-300) (Cy7) (ABIN883797)
抗原
See all SMPD1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 201-300
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交叉反应
- 人, 小鼠, 大鼠
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预测反应
- Dog,Cow,Pig,Rabbit
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human Acid sphingomyelinase
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亚型
- IgG
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anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 391-419), (C-Term) antibody
SMPD1 适用: 人 WB, FACS, IHC (p) 宿主: 兔 Polyclonal RB31029 unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 319-579) antibodySMPD1 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 70-340) antibodySMPD1 适用: 人 WB, IF 宿主: 兔 Polyclonal unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 1-364) antibodySMPD1 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 4H2 unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 391-419), (C-Term) antibodySMPD1 适用: 人 WB, FACS, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 201-300) antibodySMPD1 适用: 人, 小鼠, 大鼠 WB, ELISA, IF (cc), IF (p), ICC, IHC (p), IHC (fro) 宿主: 兔 Polyclonal unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 396-445) antibodySMPD1 适用: 人, 小鼠, 大鼠, Cow, 绵羊, 犬, 马, 兔, Pig, Bat, 猴 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1) (AA 391-419) antibodySMPD1 适用: 人 WB, IHC, FACS, IHC (p) 宿主: 兔 Polyclonal unconjugated
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应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- SMPD1 (Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1))
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别名
- Acid sphingomyelinase
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背景
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Synonyms: Acid sphingomyelinase, ASM, ASM_HUMAN, aSMase, NPD, Smpd1, Sphingomyelin phosphodiesterase 1 acid lysosomal, Sphingomyelin phosphodiesterase.
Background: 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.Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) , 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.
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基因ID
- 6609
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UniProt
- P17405
抗原
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