Single-stranded DNA-binding protein, mitochondrial

Definition:

Binds preferentially and cooperatively to pyrimidine rich single-stranded DNA (ss-DNA). In vitro, required to maintain the copy number of mitochondrial DNA (mtDNA) and plays a crucial role during mtDNA replication by stimulating the activity of the replisome components POLG and TWNK at the replication fork. Promotes the activity of the gamma complex polymerase POLG, largely by organizing the template DNA and eliminating secondary structures to favor ss-DNA conformations that facilitate POLG activity. In addition it is able to promote the 5'-3' unwinding activity of the mtDNA helicase TWNK. May also function in mtDNA repair.

References:

[1]. Marcos T Oliveira, et al. Reduced stimulation of recombinant DNA polymerase γ and mitochondrial DNA (mtDNA) helicase by variants of mitochondrial single-stranded DNA-binding protein (mtSSB) correlates with defects in mtDNA replication in animal cells. J Biol Chem. 2011 Nov 25;286(47):40649-58. [Content Brief]

[2]. Jenny A Korhonen, et al. Reconstitution of a minimal mtDNA replisome in vitro. EMBO J. 2004 Jun 16;23(12):2423-9. [Content Brief]

[3]. Valentina Del Dotto, et al. SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder. J Clin Invest. 2020 Jan 2;130(1):108-125. [Content Brief]

[4]. Jenny A Korhonen, et al. TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein. J Biol Chem. 2003 Dec 5;278(49):48627-32. [Content Brief]

[5]. Barbara van Loon, et al. Alkyladenine DNA glycosylase (AAG) localizes to mitochondria and interacts with mitochondrial single-stranded binding protein (mtSSB). DNA Repair (Amst). 2013 Mar 1;12(3):177-87. [Content Brief]

[6]. Grzegorz L Ciesielski, et al. Mitochondrial Single-stranded DNA-binding Proteins Stimulate the Activity of DNA Polymerase γ by Organization of the Template DNA. J Biol Chem. 2015 Nov 27;290(48):28697-707. [Content Brief]

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