ASPA Antibody
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中文名稱：ASPA兔多克隆抗體
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貨號：CSB-PA790667
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規格：￥1100
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圖片：
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The image on the left is immunohistochemistry of paraffin-embedded Human liver cancer tissue using CSB-PA790667(ASPA Antibody) at dilution 1/80, on the right is treated with fusion protein. (Original magnification: ×200) -
The image on the left is immunohistochemistry of paraffin-embedded Human lymphoma tissue using CSB-PA790667(ASPA Antibody) at dilution 1/80, on the right is treated with fusion protein. (Original magnification: ×200) -
Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: Mouse kidney tissue, human fetal kidney tissue, Primary antibody: CSB-PA790667(ASPA Antibody) at dilution 1/1400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 second
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：ACY 2 antibody; ACY-2 antibody; ACY2 antibody; ACY2_HUMAN antibody; Aminoacylase 2 antibody; Aminoacylase-2 antibody; Aminoacylase2 antibody; ASP antibody; ASPA antibody; Aspartoacylase (aminoacylase 2; Canavan disease) antibody; Aspartoacylase (Canavan disease) antibody; Aspartoacylase antibody; NUR 7 antibody; NUR7 antibody; OTTMUSP00000006437 antibody; RP23-213I10.1 antibody; Small lethargic antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Fusion protein of Human ASPA
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：Antigen affinity purification
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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推薦稀釋比：
Application Recommended Dilution ELISA 1:2000-1:10000 WB 1:1000-1:5000 IHC 1:100-1:300 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.
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基因功能參考文獻：
- report of 2 Egyptian sibling patients suspected of Canavan disease (CD); study revealed homozygosity for substitution T530C (Ile177Thr) in exon 4 of the ASPA gene in both sibs; substitution T530C (Ile177Thr) results in a novel missense mutation causing a CD phenotype with severe clinical characteristics PMID: 24036223
- Four ASPA missense mutations associated with Canavan disease are structurally characterized. PMID: 25003821
- Definitive evidence is presented to show that the recombinantly-expressed human aspartoacylase is not a glycoprotein. PMID: 24632142
- This is the first case report of ASPA mutation studies in Canavan disease from Indian subcontinent. PMID: 22878930
- a novel mutation Y88X within the aspartoacylase gene in a consanguineous family with an affected child diagnosed as Canavan disease. PMID: 22468686
- Human aspartoacylase gene expression was high not only in brain and kidney, but also in lung and liver. PMID: 22750302
- Gene ASPA (NM_000049) was undertaken to sequence for mutation analysis. PMID: 22219087
- We report on an Italian female patient with Canavan disease due to a missense mutation of the aspartoacylase gene and a 17p13.3 chromosomal microdeletion PMID: 22019069
- the ASPA gene was analysed in 22 unrelated non-Jewish patients with Canavan disease, and 24 different mutations were found PMID: 12638939
- Mild-onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase gene PMID: 16437572
- molecular weight of the purified enzyme is higher than predicted, suggesting the presence of post-translational modifications. Deglycosylation of aspartoacylase or mutation at glycosylation site causes decreased enzyme stability and catalytic activity PMID: 16669630
- a green fluorescent protein-human ASPA fusion protein larger than the permissible size for the nuclear pore complex was enzymatically active and showed mixed nuclear-cytoplasmic distribution. PMID: 16935940
- The finding that wild-type and Glu178Asp have the same K(m) but different k(cat) values confirms the idea that the carboxylate group contributes importantly to the enzymatic activity of aspartoacylase. PMID: 17027983
- the N-terminal domain of aspartoacylase adopts a protein fold similar to that of zinc-dependent hydrolases related to carboxypeptidases A PMID: 17194761
- These results show that aspartoacylase is a member of the caboxypeptidase A family and offer novel explanations for most loss-of-function aspartoacylase mutations associated with Canavan Disease. PMID: 17391648
- New structure of human aspartoacylase complexed with a catalytic intermediate analogue, N-phosphonomethyl- l-aspartate, supports a carboxypeptidase-type mechanism for hydrolysis of the amide bond of the substrate, N-acetyl- l-aspartate. PMID: 18293939
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相關疾病：Canavan disease (CAND)
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亞細胞定位：Cytoplasm. Nucleus.
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蛋白家族：AspA/AstE family, Aspartoacylase subfamily
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組織特異性：Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.
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數據庫鏈接：
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