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Literature summary extracted from

  • Sano, Y.; Furuta, A.; Setsuie, R.; Kikuchi, H.; Wang, Y.L.; Sakurai, M.; Kwon, J.; Noda, M.; Wada, K.
    Photoreceptor cell apoptosis in the retinal degeneration of Uchl3-deficient mice (2006), Am. J. Pathol., 169, 132-141.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
3.4.19.12 medicine Uchl3-deficient mice represent a model for adultonset retinal degeneration associated with mitochondrial impairment Mus musculus

Protein Variants

EC Number Protein Variants Comment Organism
3.4.19.12 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

EC Number Organism UniProt Comment Textmining
3.4.19.12 Mus musculus
-
-
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Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.19.12 eye level of UCH-L3 decreases with age, while the level of UCH-L1 increases with age in wild-type mice Mus musculus
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3.4.19.12 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
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Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.19.12 Nedd-8 + H2O
-
 Mus musculus ?
-
 ?
3.4.19.12 ubiquitin + H2O
-
 Mus musculus ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.19.12 UCH-L1
-
 Mus musculus
3.4.19.12 UCH-L3
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 Mus musculus