General Information of Drug Off-Target (DOT) (ID: OTXZ3YIU)

DOT Name Filensin (BFSP1)
Synonyms Beaded filament structural protein 1; Lens fiber cell beaded-filament structural protein CP 115; CP115; Lens intermediate filament-like heavy; LIFL-H
Gene Name BFSP1
Related Disease
Cataract ( )
Cataract 33 ( )
Neoplasm ( )
Early-onset nuclear cataract ( )
Cataract 31 multiple types ( )
Squamous cell carcinoma ( )
UniProt ID
BFSP1_HUMAN
3D Structure
Download
2D Sequence (FASTA)
Download
3D Structure (PDB)
Download
Pfam ID
PF00038
Sequence
MYRRSYVFQTRKEQYEHADEASRAAEPERPADEGWAGATSLAALQGLGERVAAHVQRARA
LEQRHAGLRRQLDAFQRLGELAGPEDALARQVESNRQRVRDLEAERARLERQGTEAQRAL
DEFRSKYENECECQLLLKEMLERLNKEADEALLHNLRLQLEAQFLQDDISAAKDRHKKNL
LEVQTYISILQQIIHTTPPASIVTSGMREEKLLTEREVAALRSQLEEGREVLSHLQAQRV
ELQAQTTTLEQAIKSAHECYDDEIQLYNEQIETLRKEIEETERVLEKSSYDCRQLAVAQQ
TLKNELDRYHRIIEIEGNRLTSAFIETPIPLFTQSHGVSLSTGSGGKDLTRALQDITAAK
PRQKALPKNVPRRKEIITKDKTNGALEDAPLKGLEDTKLVQVVLKEESESKFESESKEVS
PLTQEGAPEDVPDGGQISKGFGKLYRKVKEKVRSPKEPETPTELYTKERHVLVTGDANYV
DPRFYVSSITAKGGVAVSVAEDSVLYDGQVEPSPESPKPPLENGQVGLQEKEDGQPIDQQ
PIDKEIEPDGAELEGPEEKREGEERDEESRRPCAMVTPGAEEPSIPEPPKPAADQDGAEV
LGTRSRSLPEKGPPKALAYKTVEVVESIEKISTESIQTYEETAVIVETMIGKTKSDKKKS
GEKSS
Function Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA. Involved in altering the calcium regulation of MIP water permeability.
Tissue Specificity Expressed in the cortex and nucleus of the retina lens (at protein level).

Molecular Interaction Atlas (MIA) of This DOT

6 Disease(s) Related to This DOT
Disease Name Disease ID Evidence Level Mode of Inheritance REF
Cataract DISUD7SL Strong Biomarker [1]
Cataract 33 DISJ4VI5 Strong Autosomal dominant [2]
Neoplasm DISZKGEW moderate Biomarker [3]
Early-onset nuclear cataract DISGIHUY Supportive Autosomal dominant [4]
Cataract 31 multiple types DISZO5TN Limited Biomarker [5]
Squamous cell carcinoma DISQVIFL Limited Biomarker [6]
------------------------------------------------------------------------------------
⏷ Show the Full List of 6 Disease(s)
Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DOT
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 Filensin (BFSP1). [7]
Benzo(a)pyrene DMN7J43 Phase 1 Benzo(a)pyrene affects the methylation of Filensin (BFSP1). [15]
------------------------------------------------------------------------------------
9 Drug(s) Affected the Gene/Protein Processing of This DOT
Drug Name Drug ID Highest Status Interaction REF
Tretinoin DM49DUI Approved Tretinoin decreases the expression of Filensin (BFSP1). [8]
Cupric Sulfate DMP0NFQ Approved Cupric Sulfate increases the expression of Filensin (BFSP1). [9]
Hydrogen peroxide DM1NG5W Approved Hydrogen peroxide affects the expression of Filensin (BFSP1). [10]
Testosterone DM7HUNW Approved Testosterone decreases the expression of Filensin (BFSP1). [11]
Zoledronate DMIXC7G Approved Zoledronate decreases the expression of Filensin (BFSP1). [12]
Belinostat DM6OC53 Phase 2 Belinostat decreases the expression of Filensin (BFSP1). [13]
GSK2110183 DMZHB37 Phase 2 GSK2110183 increases the expression of Filensin (BFSP1). [14]
(+)-JQ1 DM1CZSJ Phase 1 (+)-JQ1 increases the expression of Filensin (BFSP1). [16]
PMID28460551-Compound-2 DM4DOUB Patented PMID28460551-Compound-2 decreases the expression of Filensin (BFSP1). [17]
------------------------------------------------------------------------------------
⏷ Show the Full List of 9 Drug(s)

References

1 Insights into the beaded filament of the eye lens.Exp Cell Res. 2007 Jun 10;313(10):2180-8. doi: 10.1016/j.yexcr.2007.04.005. Epub 2007 Apr 6.
2 Autosomal recessive juvenile onset cataract associated with mutation in BFSP1. Hum Genet. 2007 May;121(3-4):475-82. doi: 10.1007/s00439-006-0319-6. Epub 2007 Jan 16.
3 Rhabdomyosarcoma and Wilms tumors contain a subpopulation of noggin producing, myogenic cells immunoreactive for lens beaded filament proteins.PLoS One. 2019 Apr 11;14(4):e0214758. doi: 10.1371/journal.pone.0214758. eCollection 2019.
4 A novel beaded filament structural protein 1 (BFSP1) gene mutation associated with autosomal dominant congenital cataract in a Chinese family. Mol Vis. 2013 Dec 27;19:2590-5. eCollection 2013.
5 An autosomal dominant posterior polar cataract locus maps to human chromosome 20p12-q12.Eur J Hum Genet. 2000 Jul;8(7):535-9. doi: 10.1038/sj.ejhg.5200485.
6 An experimental investigation of a novel iron chelating protoporphyrin IX prodrug for the enhancement of photodynamic therapy.Lasers Surg Med. 2018 Jul;50(5):552-565. doi: 10.1002/lsm.22809. Epub 2018 Mar 31.
7 Integrative omics data analyses of repeated dose toxicity of valproic acid in vitro reveal new mechanisms of steatosis induction. Toxicology. 2018 Jan 15;393:160-170.
8 Phenotypic characterization of retinoic acid differentiated SH-SY5Y cells by transcriptional profiling. PLoS One. 2013 May 28;8(5):e63862.
9 Physiological and toxicological transcriptome changes in HepG2 cells exposed to copper. Physiol Genomics. 2009 Aug 7;38(3):386-401.
10 Global gene expression analysis reveals differences in cellular responses to hydroxyl- and superoxide anion radical-induced oxidative stress in caco-2 cells. Toxicol Sci. 2010 Apr;114(2):193-203. doi: 10.1093/toxsci/kfp309. Epub 2009 Dec 31.
11 The exosome-like vesicles derived from androgen exposed-prostate stromal cells promote epithelial cells proliferation and epithelial-mesenchymal transition. Toxicol Appl Pharmacol. 2021 Jan 15;411:115384. doi: 10.1016/j.taap.2020.115384. Epub 2020 Dec 25.
12 Interleukin-19 as a translational indicator of renal injury. Arch Toxicol. 2015 Jan;89(1):101-6.
13 Definition of transcriptome-based indices for quantitative characterization of chemically disturbed stem cell development: introduction of the STOP-Toxukn and STOP-Toxukk tests. Arch Toxicol. 2017 Feb;91(2):839-864.
14 Novel ATP-competitive Akt inhibitor afuresertib suppresses the proliferation of malignant pleural mesothelioma cells. Cancer Med. 2017 Nov;6(11):2646-2659. doi: 10.1002/cam4.1179. Epub 2017 Sep 27.
15 Air pollution and DNA methylation alterations in lung cancer: A systematic and comparative study. Oncotarget. 2017 Jan 3;8(1):1369-1391. doi: 10.18632/oncotarget.13622.
16 MCM5 as a target of BET inhibitors in thyroid cancer cells. Endocr Relat Cancer. 2016 Apr;23(4):335-47. doi: 10.1530/ERC-15-0322. Epub 2016 Feb 24.
17 Cell-based two-dimensional morphological assessment system to predict cancer drug-induced cardiotoxicity using human induced pluripotent stem cell-derived cardiomyocytes. Toxicol Appl Pharmacol. 2019 Nov 15;383:114761. doi: 10.1016/j.taap.2019.114761. Epub 2019 Sep 15.