Ubiquitin carboxyl-terminal

ZnFUBP Ubiquitin carboxyl-terminal hydrolase-like zinc finger E(MFP) 4(4) 4(4) ... 

Pickaet, C. M. and Rose, 1. A. Ubiquitin carboxyl-terminal hydrolase acts on ubiquitin carboxyl-terminal amides, J Biol Chem, 1985, 260, 7903-10. 

K. D. Ubiquitin carboxyl-terminal hydrolase (PGP 9.5) is selectively present in ubiquitinated inclusion bodies characteristic of human neurodegenerative diseases, J Pathol, 1990, 363, 153-60. 

Thiosulfate suUurtransferase Transitional endoplasmic reticulum ATPase Triosephosphate isomerase (TIM) Tropomyosin 3 Tubulin beta-1 chain Ubiquitin carboxyl-terminal hydrolase 5 Ubiquinol-cytochrome C reductase complex core protein I Vimentin Vimentin... 

Identifying altered canine and bovine proteins has proved to be particularly challenging since these species are poorly represented in current genomic databases. As a result of this, new bioinformatic tools (Multildent, http //expasy.org) have had to be developed to facilitate cross-species protein identification (Wilkins et al., 1998). The most significant change observed for bovine DCM was a sevenfold increase in the enzyme ubiquitin carboxyl-terminal hydrolase (UCH) (Weekes... 

PD is generally a sporadic disorder, but in a significant proportion of cases (10-15% in most studies) it runs in families without a clearcut Mendelian pattern. Currently, there have been 13 defined loci identified as associated with high-penetrant autosomal dominant or recessive PD, of which causative mutations in specific genes have been identified. These genes include a-synuclein, parkin, ubiquitin carboxyl-terminal esterase LI (UCH-Ll), PTEN-induced putative kinase 1 (PlNKl), DJ-1, and leucine-rich repeat kinase 2 (LRRK2). As outlined in Table 3, most of these mutations can be characterized by an early onset of disease. 

Castegna et al, 2003 Sultana et al, 2006b). Ubiquitin carboxyl-terminal hydrolase L-1 (UCH L-1), one of the components of the proteosomal pathway, was identified as an oxidized protein in the inferior parietal and hippocampal regions of AD, further suggesting a role for nitration in protein accumulation (Castegna et al., 2003 Sultana et al., 2006b). 

The PARKS-linked PD gene has been reported as the ubiquitin carboxyl-terminal hydrolase-Ll (UCHLl) located on chromosome 4pl3 (Leroy et al., 1998). UCHLl is a protein of 223 amino acids, 9 exons, and a transcript length of 1172 bps (Wilkinson et al., 1989) (Fig. 8). 

Naito S, Mochizuki H, Yasuda T, Mizuno Y, Furusaka M, Ikeda S, Adachi T, Shimizu HM, Suzuki J, Fujiwara S, Okada T, Nishikawa K, Aoki S, Wada K (2006) Chtiracterization of multimetric variants of ubiquitin carboxyl-terminal hydrolase LI in water by small-angle neutron scattering. Biochem Biophys Res Common 339 717-725... 

Nishikawa K, Li H, Kawamura R, Osaka H, Wang YL, Hara Y, Hirokawa T, Manage Y, Amano T, Noda M, Aoki S, Wada K (2003) Alterations of structure and hydrolase activity of parkinsonism-associated human ubiquitin carboxyl-terminal hydrolase LI variants. Biochem Biophys Res Commun 304 176-183... 

WiUdnson KD, Lee KM, Deshpande S, Duerksen-Hughes P, Boss JM, Pohl J (1989) The neuron-specific protein PGP 9.5 is a ubiquitin carboxyl-terminal hydrolase. Science 246 670-673 WUhams DR, Hadeed A, al-Din AS, Wreikat AL, Lees AJ (2005) Kufor Rtikeb disease autosomal recessive, levodopa-responsive parkinsonism with pyramidal degeneration, supranuclear gaze palsy, and dementia. Mov Disord 20 1264-1271... 

UCHL1 Ubiquitin carboxyl-terminal esterase LI (ubiquitin thiolesterase) 4pl4... 

UCHL3 Ubiquitin carboxyl-terminal esterase L3 (ubiquitin thiolesterase) 13q22.2... 

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