Details of Drug Off-Target (DOT)

General Information of Drug Off-Target (DOT) (ID: OT2QZKSF)

• DOT Name: Chimeric ERCC6-PGBD3 protein (ERCC6)
• Synonyms: Chimeric CSB-PGBD3 protein
• Gene Name: ERCC6
• Related Disease:
  Arthrogryposis
  Cataract
  Cockayne spectrum with or without cerebrooculofacioskeletal syndrome
  Cockayne syndrome type 2
  Herpes simplex infection
  Prostate cancer
  Prostate carcinoma
  Age-related macular degeneration
  Atrial fibrillation
  Breast cancer
  Breast carcinoma
  Cataract 20 multiple types
  Cockayne syndrome type 1
  Colon cancer
  Colon carcinoma
  Colorectal carcinoma
  Congestive heart failure
  Craniosynostosis 2
  Deafness
  Endometriosis
  Head and neck cancer
  Head and neck carcinoma
  Head-neck squamous cell carcinoma
  Hepatocellular carcinoma
  Huntington disease
  Intellectual disability
  Lung cancer
  Lung neoplasm
  Microlissencephaly
  Movement disorder
  Myelodysplastic syndrome
  Premature aging syndrome
  UV-sensitive syndrome 1
  Gastric cancer
  Isolated congenital microcephaly
  Stomach cancer
  COFS syndrome
  UV-sensitive syndrome
  Advanced cancer
  Dystonia
  Lung carcinoma
  Microphthalmia
  Neoplasm
  Nervous system disease
  Neuroblastoma
  Premature ovarian failure 11
• UniProt ID: ERPG3_HUMAN
• Pfam ID: PF13843
• Sequence:
  MPNEGIPHSSQTQEQDCLQSQPVSNNEEMAIKQESGGDGEVEEYLSFRSVGDGLSTSAVG
  CASAAPRRGPALLHIDRHQIQAVEPSAQALELQGLGVDVYDQDVLEQGVLQQVDNAIHEA
  SRASQLVDVEKEYRSVLDDLTSCTTSLRQINKIIEQLSPQAATSRDINRKLDSVKRQKYN
  KEQQLKKITAKQKHLQAILGGAEVKIELDHASLEEDAEPGPSSLGSMLMPVQETAWEELI
  RTGQMTPFGTQIPQKQEKKPRKIMLNEASGFEKYLADQAKLSFERKKQGCNKRAARKAPA
  PVTPPAPVQNKNKPNKKARVLSKKEERLKKHIKKLQKRALQFQGKVGLPKARRPWESDMR
  PEAEGDSEGEESEYFPTEEEEEEEDDEVEGAEADLSGDGTDYELKPLPKGGKRQKKVPVQ
  EIDDDFFPSSGEEAEAASVGEGGGGGRKVGRYRDDGDEDYYKQRLSPKMPRTLSLHEITD
  LLETDDSIEASAIVIQPPENATAPVSDEESGDEEGGTINNLPGSLLHTAAYLIQDGSDAE
  SDSDDPSYAPKDDSPDEVPSTFTVQQPPPSRRRKMTKILCKWKKADLTVQPVAGRVTAPP
  NDFFTVMRTPTEILELFLDDEVIELIVKYSNLYACSKGVHLGLTSSEFKCFLGIIFLSGY
  VSVPRRRMFWEQRTDVHNVLVSAAMRRDRFETIFSNLHVADNANLDPVDKFSKLRPLISK
  LNERCMKFVPNETYFSFDEFMVPYFGRHGCKQFIRGKPIRFGYKFWCGATCLGYICWFQP
  YQGKNPNTKHEEYGVGASLVLQFSEALTEAHPGQYHFVFNNFFTSIALLDKLSSMGHQAT
  GTVRKDHIDRVPLESDVALKKKERGTFDYRIDGKGNIVCRWNDNSVVTVASSGAGIHPLC
  LVSRYSQKLKKKIQVQQPNMIKVYNQFMGGVDRADENIDKYRASIRGKKWYSSPLLFCFE
  LVLQNAWQLHKTYDEKPVDFLEFRRRVVCHYLETHGHPPEPGQKGRPQKRNIDSRYDGIN
  HVIVKQGKQTRCAECHKNTTFRCEKCDVALHVKCSVEYHTE
• Function: Involved in repair of DNA damage following UV irradiation, acting either in the absence of ERCC6 or synergistically with ERCC6. Involved in the regulation of gene expression. In the absence of ERCC6, induces the expression of genes characteristic of interferon-like antiviral responses. This response is almost completely suppressed in the presence of ERCC6. In the presence of ERCC6, regulates the expression of genes involved in metabolism regulation, including IGFBP5 and IGFBP7. In vitro binds to PGBD3-related transposable elements, called MER85s; these non-autonomous 140 bp elements are characterized by the presence of PGBD3 terminal inverted repeats and the absence of internal transposase ORF.
• Tissue Specificity: Expressed in heart and oocytes, but not in granulosa cells (at protein level).

Molecular Interaction Atlas (MIA) of This DOT

46 Disease(s) Related to This DOT

Disease Name	Disease ID	Evidence Level	Mode of Inheritance	REF
Arthrogryposis	DISC81CM	Definitive	Biomarker	[1]
Cataract	DISUD7SL	Definitive	Biomarker	[1]
Cockayne spectrum with or without cerebrooculofacioskeletal syndrome	DISEQI8T	Definitive	Autosomal recessive	[2]
Cockayne syndrome type 2	DIS3X0GQ	Definitive	Autosomal recessive	[2]
Herpes simplex infection	DISL1SAV	Definitive	Altered Expression	[3]
Prostate cancer	DISF190Y	Definitive	Genetic Variation	[4]
Prostate carcinoma	DISMJPLE	Definitive	Genetic Variation	[4]
Age-related macular degeneration	DIS0XS2C	Strong	Genetic Variation	[5]
Atrial fibrillation	DIS15W6U	Strong	Biomarker	[6]
Breast cancer	DIS7DPX1	Strong	Biomarker	[7]
Breast carcinoma	DIS2UE88	Strong	Biomarker	[7]
Cataract 20 multiple types	DISN0IHS	Strong	Biomarker	[1]
Cockayne syndrome type 1	DIS9JFVY	Strong	Biomarker	[8]
Colon cancer	DISVC52G	Strong	Altered Expression	[9]
Colon carcinoma	DISJYKUO	Strong	Altered Expression	[9]
Colorectal carcinoma	DIS5PYL0	Strong	Altered Expression	[9]
Congestive heart failure	DIS32MEA	Strong	Biomarker	[6]
Craniosynostosis 2	DISBJX9D	Strong	Biomarker	[10]
Deafness	DISKCLH4	Strong	Biomarker	[11]
Endometriosis	DISX1AG8	Strong	Biomarker	[12]
Head and neck cancer	DISBPSQZ	Strong	Genetic Variation	[13]
Head and neck carcinoma	DISOU1DS	Strong	Genetic Variation	[13]
Head-neck squamous cell carcinoma	DISF7P24	Strong	Biomarker	[14]
Hepatocellular carcinoma	DIS0J828	Strong	Biomarker	[15]
Huntington disease	DISQPLA4	Strong	Biomarker	[16]
Intellectual disability	DISMBNXP	Strong	Genetic Variation	[17]
Lung cancer	DISCM4YA	Strong	Genetic Variation	[18]
Lung neoplasm	DISVARNB	Strong	Genetic Variation	[18]
Microlissencephaly	DISUCKNT	Strong	Biomarker	[1]
Movement disorder	DISOJJ2D	Strong	CausalMutation	[19]
Myelodysplastic syndrome	DISYHNUI	Strong	Genetic Variation	[20]
Premature aging syndrome	DIS51AGT	Strong	Biomarker	[11]
UV-sensitive syndrome 1	DIS0JVUT	Strong	Autosomal recessive	[21]
Gastric cancer	DISXGOUK	moderate	Altered Expression	[22]
Isolated congenital microcephaly	DISUXHZ6	moderate	Genetic Variation	[23]
Stomach cancer	DISKIJSX	moderate	Altered Expression	[22]
COFS syndrome	DISTEABI	Supportive	Autosomal recessive	[24]
UV-sensitive syndrome	DISHCN4B	Supportive	Autosomal recessive	[25]
Advanced cancer	DISAT1Z9	Limited	Altered Expression	[26]
Dystonia	DISJLFGW	Limited	Biomarker	[27]
Lung carcinoma	DISTR26C	Limited	Genetic Variation	[18]
Microphthalmia	DISGEBES	Limited	Biomarker	[28]
Neoplasm	DISZKGEW	Limited	Genetic Variation	[29]
Nervous system disease	DISJ7GGT	Limited	Genetic Variation	[30]
Neuroblastoma	DISVZBI4	Limited	Biomarker	[31]
Premature ovarian failure 11	DISD9FS5	Limited	Unknown	[32]

This DOT Affected the Drug Response of 8 Drug(s)

Drug Name	Drug ID	Highest Status	Interaction	REF
Cisplatin	DMRHGI9	Approved	Chimeric ERCC6-PGBD3 protein (ERCC6) increases the response to substance of Cisplatin.	[46]
Arsenic trioxide	DM61TA4	Approved	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of Arsenic trioxide.	[47]
Hydrogen peroxide	DM1NG5W	Approved	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of Hydrogen peroxide.	[48]
E7850	DM8K5IF	Phase 2	Chimeric ERCC6-PGBD3 protein (ERCC6) increases the response to substance of E7850.	[50]
PJ34	DMXO6YH	Preclinical	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of PJ34.	[11]
NU1025	DMPA8WO	Terminated	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of NU1025.	[11]
3-aminobenzamide	DM7P3IZ	Investigative	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of 3-aminobenzamide.	[11]
Kurarinone	DMH0G8W	Investigative	Chimeric ERCC6-PGBD3 protein (ERCC6) affects the response to substance of Kurarinone.	[47]

This DOT Affected the Regulation of Drug Effects of 4 Drug(s)

Drug Name	Drug ID	Highest Status	Interaction	REF
Nicotinamide	DMUPE07	Approved	Chimeric ERCC6-PGBD3 protein (ERCC6) increases the abundance of Nicotinamide.	[11]
Nicotinamide mononucleotide	DMW1LKP	Preclinical	Chimeric ERCC6-PGBD3 protein (ERCC6) decreases the abundance of Nicotinamide mononucleotide.	[11]
Nicotinamide-Adenine-Dinucleotide	DM9LRKB	Investigative	Chimeric ERCC6-PGBD3 protein (ERCC6) increases the metabolism of Nicotinamide-Adenine-Dinucleotide.	[11]
Acetyl Coa	DMH65Q8	Investigative	Chimeric ERCC6-PGBD3 protein (ERCC6) decreases the abundance of Acetyl Coa.	[11]

2 Drug(s) Affected the Post-Translational Modifications of This DOT

Drug Name	Drug ID	Highest Status	Interaction	REF
Valproate	DMCFE9I	Approved	Valproate decreases the methylation of Chimeric ERCC6-PGBD3 protein (ERCC6).	[33]
PMID28870136-Compound-52	DMFDERP	Patented	PMID28870136-Compound-52 increases the phosphorylation of Chimeric ERCC6-PGBD3 protein (ERCC6).	[43]

11 Drug(s) Affected the Gene/Protein Processing of This DOT

Drug Name	Drug ID	Highest Status	Interaction	REF
Doxorubicin	DMVP5YE	Approved	Doxorubicin decreases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[34]
Cupric Sulfate	DMP0NFQ	Approved	Cupric Sulfate decreases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[35]
Estradiol	DMUNTE3	Approved	Estradiol increases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[36]
Arsenic	DMTL2Y1	Approved	Arsenic affects the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[37]
Decitabine	DMQL8XJ	Approved	Decitabine increases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[36]
Cannabidiol	DM0659E	Approved	Cannabidiol decreases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[38]
Urethane	DM7NSI0	Phase 4	Urethane increases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[39]
Benzo(a)pyrene	DMN7J43	Phase 1	Benzo(a)pyrene increases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[41]
(+)-JQ1	DM1CZSJ	Phase 1	(+)-JQ1 increases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[42]
Formaldehyde	DM7Q6M0	Investigative	Formaldehyde decreases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[44]
Milchsaure	DM462BT	Investigative	Milchsaure decreases the expression of Chimeric ERCC6-PGBD3 protein (ERCC6).	[45]

1 Drug(s) Affected the Protein Interaction/Cellular Processes of This DOT

Drug Name	Drug ID	Highest Status	Interaction	REF
DNCB	DMDTVYC	Phase 2	DNCB affects the binding of Chimeric ERCC6-PGBD3 protein (ERCC6).	[40]

References

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