UVSSA:USP7 deubiquitinates ERCC6

Stable Identifier
R-HSA-6782069
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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UVSSA stabilizes ERCC6 (CSB) during transcription-coupled nucleotide excision repair (TC-NER) by targeting ubiquitin protease USP7 to ubiquitinated ERCC6, thus preventing proteasome-mediated degradation of ERCC6. Mutations in UVSSA cause UV-sensitive syndrome (Nakazawa et al. 2012, Schwertman et al. 2012, Zhang et al. 2012, Fei and Chen 2012).

Literature References
PubMed ID Title Journal Year
22466612 Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair

Zhang, X, Horibata, K, Saijo, M, Ishigami, C, Ukai, A, Kanno, S, Tahara, H, Neilan, EG, Honma, M, Nohmi, T, Yasui, A, Tanaka, K

Nat. Genet. 2012
22466611 UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair

Schwertman, P, Lagarou, A, Dekkers, DH, Raams, A, van der Hoek, AC, Laffeber, C, Hoeijmakers, JH, Demmers, JA, Fousteri, M, Vermeulen, W, Marteijn, JA

Nat. Genet. 2012
22902626 KIAA1530 protein is recruited by Cockayne syndrome complementation group protein A (CSA) to participate in transcription-coupled repair (TCR)

Fei, J, Chen, J

J. Biol. Chem. 2012
22466610 Mutations in UVSSA cause UV-sensitive syndrome and impair RNA polymerase IIo processing in transcription-coupled nucleotide-excision repair

Nakazawa, Y, Sasaki, K, Mitsutake, N, Matsuse, M, Shimada, M, Nardo, T, Takahashi, Y, Ohyama, K, Ito, K, Mishima, H, Nomura, M, Kinoshita, A, Ono, S, Takenaka, K, Masuyama, R, Kudo, T, Slor, H, Utani, A, Tateishi, S, Yamashita, S, Stefanini, M, Lehmann, AR, Yoshiura, K, Ogi, T

Nat. Genet. 2012
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
thiol-dependent ubiquitin-specific protease activity of Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1:UVSSA:USP7:XAB2 complex:XPA:TCEA1:HMGN1:EP300 [nucleoplasm]
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Orthologous Events
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