ASAH1 抗体 (AA 63-393)
Quick Overview for ASAH1 抗体 (AA 63-393) (ABIN7602206)
抗原
See all ASAH1 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 63-393
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原理
- Anti-ASAH1 Picoband® Antibody
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交叉反应 （详细）
- No cross-reactivity with other proteins.
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产品特性
- Anti-ASAH1 Picoband® Antibody (ABIN7602206). Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
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纯化方法
- Immunogen affinity purified.
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免疫原
- E.coli-derived human ASAH1 recombinant protein (Position: H63-I393).
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亚型
- IgG
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anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 144-395) antibody
ASAH1 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 25-124) antibodyASAH1 适用: 人 WB, ELISA, IHC (p) 宿主: 小鼠 Monoclonal 1A7 unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 146-395) antibodyASAH1 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 25-125) antibodyASAH1 适用: 人 WB, IHC, ELISA, IHC (p) 宿主: 小鼠 Monoclonal 1A7 unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 25-125) antibodyASAH1 适用: 人 WB, IHC (p), EIA 宿主: 小鼠 Monoclonal 1A7 unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 22-395) antibodyASAH1 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 146-395) antibodyASAH1 适用: 人 IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 1-395) antibodyASAH1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (N-Term) antibodyASAH1 适用: 人, 小鼠, 大鼠, 犬, Cow, 豚鼠, 马, Bat, Hamster, 猴 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 140-389) antibodyASAH1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
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应用备注
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Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human, Mouse
Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Alves, M. Q., Le Trionnaire, E., Ribeiro, I., Carpentier, S., Harzer, K., Levade, T., Ribeiro, M. G. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. Molec. Genet. Metab. 109: 276-281, 2013. 2. Souillet, G., Guibaud, P., Fensom, A. H., Maire, I., Zabot, M. T. Outcome of displacement bone marrow transplantation in Farber's disease: a report of a case.In: Hobbs, J. R. (ed.) : Correction of Certain Genetic Diseases by Transplantation. London: COGENT 1989. Pp. 137-141. 3. Zhou, J., Tawk, M., Tiziano, F. D., Veillet, J., Bayes, M., Nolent, F., Garcia, V., Servidei, S., Bertini, E., Castro-Giner, F., Renda, Y., Carpentier, S., Andrieu-Abadie, N., Gut, I., Levade, T., Topaloglu, H., Melki, J. Spinal muscular atrophy associated with progressive myoclonic epilepsy is caused by mutations in ASAH1. Am. J. Hum. Genet. 91: 5-14, 2012. -
限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
- 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg NaN3.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
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Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
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- ASAH1 (N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1))
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别名
- ASAH1
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背景
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Synonyms: Acid ceramidase, AC, ACDase, Acid Cdase, Acylsphingosine deacylase, N-acylethanolamine hydrolase ASAH1, N-acylsphingosine amidohydrolase, Putative 32 kDa heart protein, PHP32, Acid ceramidase subunit alpha, Acid ceramidase subunit beta, ASAH1, ASAH, HSD-33, HSD33
Tissue Specificity: Widely distributed throughout the CNS. Particularly abundant in kidney and intestinal microvilli, also detected in lung and liver. Weakly expressed in heart and aorta.
Background: The ASAH1 gene encodes in humans the acid ceramidase enzyme. It is mapped to 8p22. This gene encodes a member of the acid ceramidase family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. Processing of this preproprotein generates alpha and beta subunits that heterodimerize to form the mature lysosomal enzyme, which catalyzes the degradation of ceramide into sphingosine and free fatty acid. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. Mutations in this gene are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy.
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分子量
- 45 kDa
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基因ID
- 427
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UniProt
- Q13510
抗原
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