抗人 SSBP1 抗体对
01AB00693
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.
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技术参数
- 靶点基因名
- SSBP1
- 基因别名
- SSBP
- 蛋白别名
- single stranded DNA binding protein 1; SSBP1
- UniProt
- Q04837
- 物种
- Human
产品详情
- 靶点名称
- SSBP1
- 蛋白名称
- single stranded DNA binding protein 1
- 蛋白别名
- Single-stranded DNA-binding protein; mitochondrial; Mt-SSB; MtSSB; PWP1-interacting protein 17
- 靶点基因名
- SSBP1
- 基因别名
- SSBP
- UniProt
- Q04837
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.