E3 Ubiquitin Ligase APC/C
Anaphase-Promoting Complex-Cyclosome
/ metabolism
Carcinoma, Hepatocellular
/ metabolism
Enzyme Stability
HEK293 Cells
Half-Life
Humans
Liver Neoplasms
/ metabolism
Phenylalanine
/ metabolism
Phenylalanine Hydroxylase
/ metabolism
Polyubiquitin
/ metabolism
Proteasome Endopeptidase Complex
/ metabolism
Protein Binding
Proteolysis
Ubiquitin-Protein Ligases
/ metabolism
Ubiquitination
enzyme assay
hyperphenylalaninemia
liver cancer
neurological damage
tetrahydrobiopterin
ubiquitin-proteasome system
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
28 Nov 2020
28 Nov 2020
Historique:
received:
19
10
2020
revised:
18
11
2020
accepted:
25
11
2020
entrez:
2
12
2020
pubmed:
3
12
2020
medline:
4
3
2021
Statut:
epublish
Résumé
Phenylketonuria (PKU) is an autosomal recessive metabolic disorder caused by the dysfunction of the enzyme phenylalanine hydroxylase (PAH). Alterations in the level of PAH leads to the toxic accumulation of phenylalanine in the blood and brain. Protein degradation mediated by ubiquitination is a principal cellular process for maintaining protein homeostasis. Therefore, it is important to identify the E3 ligases responsible for PAH turnover and proteostasis. Here, we report that anaphase-promoting complex/cyclosome-Cdh1 (APC/C)
Identifiants
pubmed: 33260674
pii: ijms21239076
doi: 10.3390/ijms21239076
pmc: PMC7729981
pii:
doi:
Substances chimiques
Polyubiquitin
120904-94-1
Phenylalanine
47E5O17Y3R
Phenylalanine Hydroxylase
EC 1.14.16.1
Anaphase-Promoting Complex-Cyclosome
EC 2.3.2.27
Ubiquitin-Protein Ligases
EC 2.3.2.27
Proteasome Endopeptidase Complex
EC 3.4.25.1
ATP dependent 26S protease
EC 3.4.99.-
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : National Research Foundation of Korea
ID : 2018M3A9H3022412
Déclaration de conflit d'intérêts
The authors declare no competing interests.
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