Reactome: A Curated Pathway Database
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Pathways (5) Reactions (6) Proteins (6) Others (3)
Protein: UniProt:Q00597 FANCC (Homo sapiens)
Last changed: 2015-03-10 08:59:22

Pathway: DNA Repair (Homo sapiens)
DNA repair is a phenomenal multi-enzyme, multi-pathway system required to ensure the integrity of the cellular genome. These cellular mechanisms that must cope with the plethora of DNA base pair adducts that arise. DNA damage can arise spontaneously in the cellular milieu through chemical alteration of base nucleotides or as a consequence of errors during DNA replication. For example, it is well k
Last changed: 2015-03-06 23:15:47

Pathway: Fanconi Anemia pathway (Homo sapiens)
Fanconi anemia (FA) is a genetic disease of genome instability characterized by congenital skeletal ... tion groups correspond to the genes FANCA, FANCB, FANCC, FANCD1/BRCA2, FANCD2, FANCE, FANCF, FANCG, FANCJ/BRIP1, FANCL, FANCM, and FANCN/PALB2. Although the functions of most of these proteins are mostly unknown, eight of them, FANCA, FANCB, FANCC, FANCE, FANCF, FANCG, FANCL, and FANCM products toge ...
Last changed: 2015-03-06 23:15:47

Pathway: DNA Damage Bypass (Homo sapiens)
In addition to various processes for removing lesions from the DNA, cells have developed specific mechanisms for tolerating unrepaired damage during the replication of the genome. These mechanisms are collectively called DNA damage bypass pathways. The Y family of DNA polymerases plays a key role in DNA damage bypass. Y family DNA polymerases, REV1, POLH (DNA polymerase eta), POLK (DNA polymerase ka
Last changed: 2015-03-06 23:15:47

Pathway: Regulation of the Fanconi anemia pathway (Homo sapiens)
The Fanconi anemia DNA repair pathway is negatively regulated by the deubiquitination of FANCD2 an postively regulated by phosphorylation of the FANCD2 and FANCI. The USP1 deubiquitinating enzyme is responsible for FANCD2 deubiquitination and is activated by the WD40-repeat containing UAF1 protein through formation of a stable USP1/UAF1 protein complex (Cohn et al., 2007). ATR and ATM dependent phos
Last changed: 2015-03-06 23:15:47

Pathway: Recognition of DNA damage by PCNA-containing replication complex (Homo sapiens)
Damaged double strand DNA (dsDNA) cannot be successfully used as a template by replicative DNA polymerase delta (POLD) and epsilon (POLE) complexes (Hoege et al. 2002). When the replication complex composed of PCNA, RPA, RFC and POLD or POLE stalls at a DNA damage site, PCNA becomes monoubiquitinated by RAD18 bound to UBE2B (RAD6). POLD or POLE dissociate from monoubiquitinated PCNA, while Y family DNA
Last changed: 2015-03-06 23:15:47

Reaction: USP1 autocleavage (Homo sapiens)
UV radiation, through an unknown mechanism, triggers USP1 autocleavage immediately after a conserved Gly-Gly motif. The products of USP1 autocleavage are targeted for proteasome-mediated degradation, thus preventing the activity of USP1:ZBTB32 deubiquitinase complex and allowing for monoubiquitinated PCNA to accumulate and stimulate translesion DNA synthesis (TLS) (Huang et al. 2006)
Last changed: 2015-03-06 10:40:16

Reaction: FANCD2 deubiquitination by USP1/UAF1 (Homo sapiens)
The FA pathway is negatively regulated through the USP1/UAF1 mediated deubiquitination of FANCD2 (Nijman et al., 2005) UAF1 forms a complex with and activates USP1 (Cohn et al., 2007)
Last changed: 2015-03-06 10:40:16

Reaction: Monoubiquitination of FANCD2 by the FA ubiquitin ligase complex (Homo sapiens)
Lysine 561 of FANCD2 is monoubiquitinated by FANCL, a component of the FA core complex with ubiquitin ligase activity (Garcia Higuera et al., 2001). UBE2T binds to FANCL, and is required for the monoubiquitination of FANCD2 (Machida et al., 2006). Monoubiquitination of FANCD2 in turn is necessary for its localization to chromatin and for the formation of nuclear foci in response to DNA damage. FANCD2
Last changed: 2015-03-06 10:40:16

Reaction: USP1:ZBTB32 deubiquitinates monoUb:K164-PCNA (Homo sapiens)
Deubiquitinating enzyme USP1, bound to its accessory protein ZBTB32 (UAF1), deubiquitinates PCNA (MonoUb:K164-PCNA), thus preventing excessive activation of DNA translesion synthesis (TLS) (Huang et al. 2006)
Last changed: 2015-03-06 10:40:16

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