aspartoacylase

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81 relevant articles (2 outcomes, 5 trials/studies) found for this Bio-Agent

Description: deficiency results in CANAVAN DISEASE

Also Known As:

N-acyl-L-aspartate amidohydrolase; aminoacylase II

Relationship Network

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1

Experts

1.	Serikawa, Tadao: 4 articles (04/2007 - 08/2002)
2.	Leone, P: 4 articles (09/2006 - 07/2000)
3.	Matalon, Reuben: 3 articles (04/2007 - 05/2003)
4.	Surendran, Sankar: 3 articles (04/2007 - 05/2003)
5.	Kitada, Kazuhiro: 3 articles (04/2007 - 08/2002)
6.	Sasa, Masashi: 3 articles (04/2007 - 08/2002)
7.	Pastores, G M: 2 articles (09/2006 - 11/2002)
8.	Raghavan, S S: 2 articles (09/2006 - 04/2005)
9.	Kolodny, E H: 2 articles (09/2006 - 11/2002)
10.	Wang, Z H: 2 articles (09/2006 - 11/2002)

Related Diseases

1.	Canavan Disease (Spongy Degeneration of Infancy) 09/15/2002 - "Recent studies have shown that aspartoacylase (ASPA), the defective enzyme in Canavan disease, is detectable in the brain only in the oligodendrocytes" 09/01/2008 - "PURPOSE: Canavan disease, caused by a deficiency of aspartoacylase, is one of the most common cerebral degenerative diseases of infancy" 06/01/2008 - "Canavan disease is caused by a genetic mutation in aspartoacylase gene" 06/01/2008 - "Novel mutation of aspartoacylase gene in a Turkish patient with Canavan disease." 05/07/2007 - "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." Order ALL the reference details at left...
2.	Seizures (Seizure) 07/01/2004 - "Adenoviral gene transfer of aspartoacylase ameliorates tonic convulsions of spontaneously epileptic rats." 04/01/2007 - "Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice." 07/01/2004 - "Our previous studies have demonstrated that absence-like seizures of the tremor rat (tm/tm), one of the parent strains of SER, were inhibited by adenoviral transfer of the aspartoacylase (ASPA) gene, a deleted gene in the tremor rat" 04/27/2005 - "The tremor rat is a spontaneous epilepsy model with a seizure phenotype caused by a deletion in the aspartoacylase (ASPA) gene" 08/01/2007 - "This inherited metabolic disease, caused by deficiency of the enzyme aspartoacylase, is clinically characterized by severe mental retardation, hypotonia and macrocephaly, and also generalized tonic and clonic type seizures in about half of the patients" Order ALL the reference details at left...
3.	Nervous System Diseases (Neurological Disorders) 07/01/2000 - "With the ultimate goal of developing safe and effective in vivo gene therapy for the treatment of Canavan disease and other neurological disorders, we developed a non-viral lipid-entrapped, polycation-condensed delivery system (LPD) for central nervous system gene transfer, in conjunction with adeno-associated virus (AAV)-based plasmids containing recombinant aspartoacylase (ASPA)" 03/18/2008 - "Canavan disease is a fatal neurological disorder caused by the malfunctioning of a single metabolic enzyme, aspartoacylase, that catalyzes the deacetylation of N-acetyl-L-aspartate to produce L-aspartate and acetate" Order ALL the reference details at left...
4.	Tremor (Tremors) 07/01/2006 - "We present here a developmental analysis of aspartoacylase (Aspa)-null tremor rats and show a potential role for this hydrolytic enzyme in the regulation of a postnatal neurotrophic stimulus that impacts on early stages of oligodendrocyte differentiation" 04/27/2005 - "This study provides evidence that AAV-2-mediated aspartoacylase gene transfer to the brain improves biochemical and behavioral deficits in tremor rat mutants (tm/tm) and supports the rationale of human gene transfer for Canavan disease." 07/01/2004 - "Our previous studies have demonstrated that absence-like seizures of the tremor rat (tm/tm), one of the parent strains of SER, were inhibited by adenoviral transfer of the aspartoacylase (ASPA) gene, a deleted gene in the tremor rat" 08/16/2002 - "Adenoviral gene transfer of aspartoacylase into the tremor rat, a genetic model of epilepsy, as a trial of gene therapy for inherited epileptic disorder." 04/27/2005 - "Effects of AAV-2-mediated aspartoacylase gene transfer in the tremor rat model of Canavan disease." Order ALL the reference details at left...
5.	Epilepsy (Aura) 08/16/2002 - "Adenoviral gene transfer of aspartoacylase into the tremor rat, a genetic model of epilepsy, as a trial of gene therapy for inherited epileptic disorder." 04/27/2005 - "The tremor rat is a spontaneous epilepsy model with a seizure phenotype caused by a deletion in the aspartoacylase (ASPA) gene" 09/01/2003 - "PURPOSE: We previously reported that an increase in the N-acetyl-L-aspartate (NAA) level due to the lack of aspartoacylase gene was found in the brain of the tremor rat (tm/tm), which is a mutant with a causative gene named tm that shows epileptic seizures" Order ALL the reference details at left...

Related Drugs and Biologics

1.	Biological Markers (Surrogate Marker)
2.	N-acetylaspartate (N-acetyl aspartate)
3.	Aspartic Acid (Aspartate)
4.	DNA (Deoxyribonucleic Acid)
5.	aspartoacylase
6.	Apolipoprotein C-III
7.	A-Form DNA (A-DNA)
8.	Intercellular Adhesion Molecule-1 (Intercellular Adhesion Molecule 1)
9.	Calcitriol Receptors (Calcitriol Receptor)
10.	Complementary DNA (cDNA)

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