PUBLICATION
            Cloning and expression analysis of a Parkinson's disease gene, uch-L1, and its promoter in zebrafish
- Authors
- Son, O.-L., Kim, H.-T., Ji, M.-H., Yoo, K.-W., Rhee, M., and Kim, C.-H.
- ID
- ZDB-PUB-040113-8
- Date
- 2003
- Source
- Biochemical and Biophysical Research Communications 312(3): 601-607 (Journal)
- Registered Authors
- Ji, Mi-Hyun, Kim, Cheol-Hee, Kim, Hyun-Taek, Son, Ok-Lye, Yoo, Kyeong-Won
- Keywords
- none
- MeSH Terms
- 
    
        
        
            
                - Mice
- Gene Expression Profiling/methods*
- Cloning, Molecular
- Molecular Sequence Data
- Gene Expression Regulation, Developmental*
- Amino Acid Sequence
- Humans
- Gene Expression Regulation, Enzymologic*
- Sequence Homology
- Animals
- Species Specificity
- Tissue Distribution
- Zebrafish Proteins/chemistry
- Zebrafish Proteins/genetics*
- Zebrafish Proteins/metabolism*
- Promoter Regions, Genetic/genetics
- Ubiquitin Thiolesterase/chemistry
- Ubiquitin Thiolesterase/genetics*
- Ubiquitin Thiolesterase/metabolism*
- Zebrafish/embryology
- Zebrafish/genetics*
- Zebrafish/metabolism*
- Models, Animal
- Parkinson Disease/genetics*
- Parkinson Disease/metabolism*
 
- PubMed
- 14680807 Full text @ Biochem. Biophys. Res. Commun.
            Citation
        
        
            Son, O.-L., Kim, H.-T., Ji, M.-H., Yoo, K.-W., Rhee, M., and Kim, C.-H. (2003) Cloning and expression analysis of a Parkinson's disease gene, uch-L1, and its promoter in zebrafish. Biochemical and Biophysical Research Communications. 312(3):601-607.
        
    
                
                    
                        Abstract
                    
                    
                
                
            
        
        
    
        
            
            
 
    
    
        
    
    
    
        
                Three genes, alpha-synuclein, parkin, and ubiquitin C-terminal hydrolase L1 (UCH-L1), have been associated with inherited forms of Parkinson's disease (PD), although their in vivo functions have remained largely unknown. To develop an animal model for the molecular study of PD, we cloned zebrafish uch-L1 cDNA and its gene promoter. Sequence analysis revealed that the zebrafish Uch-L1 is highly homologous (79%) to the human UCH-L1, which is a member of the deubiquitinating enzymes. By whole-mount in situ hybridization, we examined the spatiotemporal expression of uch-L1 mRNA in developing zebrafish embryos. The uch-L1 mRNAs are detected in neuronal cells at the first day of embryo development. The expression domain of uch-L1 overlaps with that of tyrosine hydroxylase, a molecular marker for dopaminergic neurons, in the ventral diencephalon, an equivalent structure to the substantia nigra where PD progresses in human. To further analyze the tissue-specific regulation of uch-L1 gene expression, we also tested its gene promoter activity and showed a preferential neuronal expression in transient transgenic zebrafish embryos. These results suggest that uch-L1 may have an important role in the development of neuronal cells in early embryos as well as in the degeneration and disease of neuronal cells in late adult brain.
            
    
        
        
    
    
    
                
                    
                        Genes / Markers
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Expression
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Phenotype
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Mutations / Transgenics
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Human Disease / Model
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Sequence Targeting Reagents
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Fish
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Orthology
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Engineered Foreign Genes
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    
                
                    
                        Mapping
                    
                    
                
                
            
        
        
    
        
            
            
        
        
    
    
    