UVSSA:USP7 deubiquitinates ERCC6

Stable Identifier
Reaction [transition]
Homo sapiens
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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
22466611 UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair

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

Nat. Genet. 2012
22466612 Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair

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

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

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

Nat. Genet. 2012
Catalyst Activity

cysteine-type deubiquitinase 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]

Orthologous Events
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