ASPA Antibody

說明書

中文名稱：

ASPA兔多克隆抗體
貨號：

CSB-PA002223GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P45381
基因名：

ASPA
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
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human ASPA
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
1. 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
2. Four ASPA missense mutations associated with Canavan disease are structurally characterized. PMID: 25003821
3. Definitive evidence is presented to show that the recombinantly-expressed human aspartoacylase is not a glycoprotein. PMID: 24632142
4. This is the first case report of ASPA mutation studies in Canavan disease from Indian subcontinent. PMID: 22878930
5. a novel mutation Y88X within the aspartoacylase gene in a consanguineous family with an affected child diagnosed as Canavan disease. PMID: 22468686
6. Human aspartoacylase gene expression was high not only in brain and kidney, but also in lung and liver. PMID: 22750302
7. Gene ASPA (NM_000049) was undertaken to sequence for mutation analysis. PMID: 22219087
8. 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
9. the ASPA gene was analysed in 22 unrelated non-Jewish patients with Canavan disease, and 24 different mutations were found PMID: 12638939
10. Mild-onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase gene PMID: 16437572
11. 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
12. 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
13. 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
14. the N-terminal domain of aspartoacylase adopts a protein fold similar to that of zinc-dependent hydrolases related to carboxypeptidases A PMID: 17194761
15. 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
16. 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)
亞細胞定位：

Cytoplasm. Nucleus.
蛋白家族：

AspA/AstE family, Aspartoacylase subfamily
組織特異性：

Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.
數據庫鏈接：

HGNC: 756

OMIM: 271900

KEGG: hsa:443

STRING: 9606.ENSP00000263080

UniGene: Hs.171142

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