TOPBP1

TOPBP1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1WF6, 2XNH, 2XNK, 3AL2, 3AL3, 3JVE, 3OLC, 3PD7, 3UEN, 3UEO
Identifiers
Aliases	TOPBP1, TOP2BP1, topoisomerase (DNA) II binding protein 1, DNA topoisomerase II binding protein 1, Dpb11
External IDs	OMIM: 607760; MGI: 1920018; HomoloGene: 38262; GeneCards: TOPBP1; OMA:TOPBP1 - orthologs
Gene location (Human)
Chr.	Chromosome 3 (human)
End	133,662,380 bp
Gene location (Mouse)
Chr.	Chromosome 9 (mouse)
End	103,227,627 bp
RNA expression pattern
	Top expressed in
	sperm; ; ventricular zone; ; ganglionic eminence; ; bone marrow; ; bone marrow cells; ; rectum; ; Achilles tendon; ; appendix; ; monocyte; ; lymph node;
	Top expressed in
	tail of embryo; ; otic placode; ; genital tubercle; ; abdominal wall; ; saccule; ; otic vesicle; ; primitive streak; ; dermis; ; mandibular prominence; ; ventricular zone;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	DNA binding; protein C-terminus binding; protein binding; identical protein binding; isomerase activity;
Cellular component	PML body; spindle pole; male germ cell nucleus; nucleoplasm; microtubule organizing center; chromosome; condensed nuclear chromosome; actin cytoskeleton; cytoskeleton; nucleus; cytoplasm; nuclear body;
Biological process	response to ionizing radiation; DNA metabolic process; cellular response to DNA damage stimulus; DNA repair; DNA replication initiation; mitotic G2 DNA damage checkpoint signaling; mitotic DNA replication checkpoint signaling; DNA replication; regulation of signal transduction by p53 class mediator;
	Sources:Amigo / QuickGO
Orthologs
	11073
	235559
	ENSG00000163781
	ENSMUSG00000032555
	Q92547
	Q6ZQF0
	NM_007027; NM_001363889
	NM_176979
	NP_008958; NP_001350818; NP_008958.2
	NP_795953
	Wikidata
View/Edit Human	View/Edit Mouse

DNA topoisomerase 2-binding protein 1 (TOPBP1) is a scaffold protein that in humans is encoded by the TOPBP1 gene.

TOPBP1 was first identified as a protein binding partner of DNA topoisomerase-IIβ by a yeast 2-hybrid screen, giving it its name. TOPBP1 is involved in a variety of nuclear specific events. These include DNA damage repair, DNA replication, transcriptional regulation, and cell cycle checkpoint activation. TOPBP1 primarily regulates the DNA damage repair response through its ability to activate the damage response kinase, ataxia-telangiectasia mutated and RAD3-related (ATR). It also plays a critical role in DNA replication initiation and regulation of the cell cycle. Changes in TOPBP1 gene expression are associated with pulmonary hypertension, breast cancer, glioblastoma, non-small cell lung cancer, and sarcomas.