FBH1
entrez
84893
canonical
uniprot
Q8NFZ0
canonical
ensembl
ENSG00000134452
resolver
ensp
ENSP00000355415
resolver
ensp
ENSP00000369335
resolver
ensp
ENSP00000380439
resolver
ensp
ENSP00000677494
resolver
enst
ENST00000397269
resolver
genesymbol
FBH1
resolver
genesymbol
FBXO18
resolver
genesymbol_syn
FBX18
resolver
genesymbol_syn
FBXO18
resolver
hgnc
HGNC:13620
resolver
uniprot
B3KV95
resolver
uniprot
F6UZG9
resolver
uniprot
Q2TAK1
resolver
uniprot
Q5JVB0
resolver
uniprot
Q5JVB1
resolver
uniprot
Q7Z4Q6
resolver
uniprot
Q7Z4R0
resolver
uniprot
Q8N1P5
resolver
uniprot
Q8N586
resolver
uniprot
Q96E82
resolver
uniprot
Q96K67
resolver
uniprot
Q96SW7
resolver
uniprot
Q9UFB2
resolver
uniprot_entry
B3KV95_HUMAN
resolver
uniprot_entry
F6UZG9_HUMAN
resolver
uniprot_entry
FBH1_HUMAN
resolver
uniprot_entry
Q2TAK1_HUMAN
resolver
uniprot_sec
Q5JVB0
resolver
uniprot_sec
Q5JVB1
resolver
uniprot_sec
Q7Z4Q6
resolver
uniprot_sec
Q7Z4R0
resolver
uniprot_sec
Q8N1P5
resolver
uniprot_sec
Q8N586
resolver
uniprot_sec
Q96E82
resolver
uniprot_sec
Q96K67
resolver
uniprot_sec
Q96SW7
resolver
uniprot_sec
Q9UFB2
resolver
amino_acid_sequence
MRRFKRKHLTAIDCQHLARSHLAVTQPFGQRWTNRDPNHGLYPKPRTKRGSRGQGSQRCIPEFFLAGKQPCTNDMAKSNSVGQDSCQDSEGDMIFPAESSCALPQEGSAGPGSPGSAPPSRKRSWSSEEESNQATGTSRWDGVSKKAPRHHLSVPCTRPREARQEAEDSTSRLSAESGETDQDAGDVGPDPIPDSYYGLLGTLPCQEALSHICSLPSEVLRHVFAFLPVEDLYWNLSLVCHLWREIISDPLFIPWKKLYHRYLMNEEQAVSKVDGILSNCGIEKESDLCVLNLIRYTATTKCSPSVDPERVLWSLRDHPLLPEAEACVRQHLPDLYAAAGGVNIWALVAAVVLLSSSVNDIQRLLFCLRRPSSTVTMPDVTETLYCIAVLLYAMREKGINISNRIHYNIFYCLYLQENSCTQATKVKEEPSVWPGKKTIQLTHEQQLILNHKMEPLQVVKIMAFAGTGKTSTLVKYAEKWSQSRFLYVTFNKSIAKQAERVFPSNVICKTFHSMAYGHIGRKYQSKKKLNLFKLTPFMVNSVLAEGKGGFIRAKLVCKTLENFFASADEELTIDHVPIWCKNSQGQRVMVEQSEKLNGVLEASRLWDNMRKLGECTEEAHQMTHDGYLKLWQLSKPSLASFDAIFVDEAQDCTPAIMNIVLSQPCGKIFVGDPHQQIYTFRGAVNALFTVPHTHVFYLTQSFRFGVEIAYVGATILDVCKRVRKKTLVGGNHQSGIRGDAKGQVALLSRTNANVFDEAVRVTEGEFPSRIHLIGGIKSFGLDRIIDIWILLQPEEERRKQNLVIKDKFIRRWVHKEGFSGFKRYVTAAEDKELEAKIAVVEKYNIRIPELVQRIEKCHIEDLDFAEYILGTVHKAKGLEFDTVHVLDDFVKVPCARHNLPQLPHFRVESFSEDEWNLLYVAVTRAKKRLIMTKSLENILTLAGEYFLQAELTSNVLKTGVVRCCVGQCNNAIPVDTVLTMKKLPITYSNRKENKGGYLCHSCAEQRIGPLAFLTASPEQVRAMERTVENIVLPRHEALLFLVF
uniprot
proteins
disease_involvement
DISEASE: Note=Defects in FBH1 are frequently observed in melanomas, resulting in increased survival in response to replicative stress. Its inactivation may play a role in oncogenic transformation. {ECO:0000269|PubMed:23466708}.
uniprot
proteins
ec_number
5.6.2.4
uniprot
proteins
function
FUNCTION: 3'-5' DNA helicase and substrate-recognition component of the SCF(FBH1) E3 ubiquitin ligase complex that plays a key role in response to stalled/damaged replication forks (PubMed:11956208, PubMed:23393192). Involved in genome maintenance by acting as an anti-recombinogenic helicase and preventing extensive strand exchange during homologous recombination: promotes RAD51 filament dissolution from stalled forks, thereby inhibiting homologous recombination and preventing excessive recombination (PubMed:17724085, PubMed:19736316). Also promotes cell death and DNA double-strand breakage in response to replication stress: together with MUS81, promotes the endonucleolytic DNA cleavage following prolonged replication stress via its helicase activity, possibly to eliminate cells with excessive replication stress (PubMed:23319600, PubMed:23361013). Plays a major role in remodeling of stalled DNA forks by catalyzing fork regression, in which the fork reverses and the two nascent DNA strands anneal (PubMed:25772361). In addition to the helicase activity, also acts as the substrate-recognition component of the SCF(FBH1) E3 ubiquitin ligase complex, a complex that mediates ubiquitination of RAD51, leading to regulate RAD51 subcellular location (PubMed:25585578). {ECO:0000269|PubMed:11956208, ECO:0000269|PubMed:17724085, ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23319600, ECO:0000269|PubMed:23361013, ECO:0000269|PubMed:25585578, ECO:0000269|PubMed:25772361}.
uniprot
proteins
mass_dalton
117686
uniprot
proteins
mutagenesis
MUTAGEN 56; /note="S->A: No effect; when associated with A-583."; /evidence="ECO:0000269|PubMed:23319600"; MUTAGEN 60..64; /note="IPEFF->APAAA: In PIPdeg3A; reduced ubiquitination."; /evidence="ECO:0000269|PubMed:23677613"; MUTAGEN 63..64; /note="FF->AA: Impaired localization to DNA damage sites in response to UV irradiation."; /evidence="ECO:0000269|PubMed:23677613"; MUTAGEN 227..228; /note="LP->AA: Impairs formation of the SCF(FBH1) complex and impairs accumulation on single-stranded DNA."; /evidence="ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23319600"; MUTAGEN 583; /note="S->A: No effect; when associated with A-56."; /evidence="ECO:0000269|PubMed:23319600"; MUTAGEN 647; /note="D->N: Abolishes helicase activity and prevents accumulation on single-stranded DNA."; /evidence="ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23319600, ECO:0000269|PubMed:25772361"; MUTAGEN 807..809; /note="KFI->AAA: Impaired localization to DNA damage sites in response to UV irradiation."; /evidence="ECO:0000269|PubMed:23677613
uniprot
proteins
pathway_participation
PATHWAY: Protein modification; protein ubiquitination. {ECO:0000269|PubMed:25585578}.
uniprot
proteins
post_translational_modification
PTM: Ubiquitinated (PubMed:23393192, PubMed:23677613). Ubiquitination by the DCX(DTL) complex, also named CRL4(CDT2), leading to its degradation: ubiquitination takes place after its localization to DNA damage sites, possibly to facilitate the translesion synthesis (TLS) pathway (PubMed:23677613). {ECO:0000269|PubMed:23393192, ECO:0000269|PubMed:23677613}.
uniprot
proteins
protein_family
Helicase family
uniprot
proteins
protein_family
UvrD subfamily
uniprot
proteins
pubmed
11956208
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15164054
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
17724085
uniprot
proteins
pubmed
17974005
uniprot
proteins
pubmed
19736316
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
23319600
uniprot
proteins
pubmed
23361013
uniprot
proteins
pubmed
23393192
uniprot
proteins
pubmed
23466708
uniprot
proteins
pubmed
23677613
uniprot
proteins
pubmed
25585578
uniprot
proteins
pubmed
25772361
uniprot
proteins
sequence_length
1043
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Nucleus {ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23677613}. Chromosome {ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23319600, ECO:0000269|PubMed:23677613}. Note=Accumulates at sites of DNA damage or replication stress (PubMed:19736316, PubMed:23677613). PCNA is required for localization to DNA damage sites (PubMed:23677613). Localizes to the nucleoplasm in absence of DNA damage (PubMed:23677613). {ECO:0000269|PubMed:19736316, ECO:0000269|PubMed:23677613}.
uniprot
proteins