Anti-Human SSBP1 Antibody Pair

Product Description: Single stranded DNA binding protein 1 (SSBP1) is a crucial protein involved in mitochondrial DNA replication, maintenance, and repair. It binds to single-stranded DNA (ssDNA) to prevent secondary structures that could impede downstream replication or repair processes. The protein is a tetramer that plays a pivotal role in stabilizing ssDNA intermediates formed during replication, recombination, and repair reactions. Mutations in the SSBP1 gene have been linked to a range of mitochondrial disorders, including optic atrophy, retinal degeneration, and Leigh syndrome, characterized by muscle weakness, seizures, hearing loss, and developmental delays. The protein's structure, featuring two distinct DNA binding sites, has been elucidated through X-ray crystallography, highlighting its critical role in mitochondrial genome integrity. Protein Function: Binds preferentially and cooperatively to pyrimidine rich single-stranded DNA (ss-DNA) (PubMed:21953457, PubMed:23290262, PubMed:31550240). 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 (PubMed:12975372, PubMed:15167897, PubMed:21953457, PubMed:26446790, PubMed:31550240). 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 (PubMed:21953457, PubMed:26446790, PubMed:31550240). In addition it is able to promote the 5'-3' unwinding activity of the mtDNA helicase TWNK (PubMed:12975372). May also function in mtDNA repair (PubMed:23290262) [The Uniprot Consortium]