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Address: 14th Floor, Building A, Wuhan Guoying Seed Industry Building, No. 666, Shendun Fourth Road, Jiangxia District, Wuhan, Hubei Province
Email: pinuofei2017@163.com
TEL: 15392937510
Article Number: PL0424
Delivery time: 现货
Price: 50 μL/960; 100 μL/1600; 200 μL/2560
Category:
Target： ASPA
Application： WB, IHC, IF, IP, ELISA
Reactivity ： Human,Mouse,Rat
MW(CalcμLated) ： 36 kD
MW(Observed) ： 33 kDa
Host Species： Rabbit
Isotype ： IgG, Kappa
隐藏域元素占位
Detailed Information
Recommended dilution ratio ： IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200;
Compose ： PBS, 50% glycerol, 0.05% Proclin 300, 0.05% BSA
Purification process ： Protein A
Storage ： -15°C to -25°C/1 year(Do not lower than -25°C
Clonality ： Monoclonal
Clone Number ： PT1339R
Antigen & Target Information
Specificity ： Endogenous
Gene name ： ASPA ACY2 ASP
Protein Name ： ACY2
Database connection:
Background：
This gene encodes an enzyme that catalyzes the conversion of N-acetyl_L-aspartic acid (NAA) to aspartate and acetate. NAA is abundant in the brain where hydrolysis by aspartoacylase is thought to help maintain white matter. This protein is an NAA scavenger in other tissues. Mutations in this gene cause Canavan disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008],
Cell localization ： Catalytic activity: N-acyl-L-aspartate + H(2)O = a carboxylate + L-aspartate. Cofactor: Binds 1 zinc ion per subunit. Disease: Defects in ASPA are the cause of Canavan disease (CAND) [MIM:271900]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demyelination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average. Function: 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. Similarity: Belongs to the aspA/astE family. Aspartoacylase subfamily. Subunit: Homodimer. Tissue specificity: Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.
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Address: 14th Floor, Building A, Wuhan Guoying Seed Industry Building, No. 666, Shendun Fourth Road, Jiangxia District, Wuhan, Hubei Province
Email: pinuofei2017@163.com
TEL: 15392937510
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