Application | Comment | Organism |
---|---|---|
medicine | Uchl3-deficient mice represent a model for adultonset retinal degeneration associated with mitochondrial impairment | Mus musculus |
Protein Variants | Comment | Organism |
---|---|---|
additional information | Uchl3 deletion mutant displays retinal degeneration, muscular degeneration, and mild growth retardation. No significant morphological abnormalities during retinal development, prominent retinal degeneration becomes manifested after 3 weeks of age associated with photoreceptor cell apoptosis. Decreased area of mitochondrial cristae and vacuolar changes in the degenerated inner segment. Loss of UCH-L3 leads to mitochondrial oxidative stress-related photoreceptor cell apoptosis in a caspase-independent manner | Mus musculus |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
eye | level of UCH-L3 decreases with age, while the level of UCH-L1 increases with age in wild-type mice | Mus musculus | - |
retina | localization of UCH-L3 in the wild-type retina is altered with age. UCH-L3 is enriched in the photoreceptor inner segment that contains abundant mitochondria. UCH-L1 is expressed in both genotypes in the inner retina, which consists of the inner nuclear layer, inner plexiform layer, and ganglion cell layer | Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
Nedd-8 + H2O | - |
Mus musculus | ? | - |
? | |
ubiquitin + H2O | - |
Mus musculus | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
UCH-L1 | - |
Mus musculus |
UCH-L3 | - |
Mus musculus |