人 DBR1 抗体对
01AB02064
Debranching RNA lariats 1 (DBR1) is an evolutionarily conserved protein that acts as the sole enzyme responsible for hydrolyzing the 2′–5′ phosphodiester bond in lariat RNAs, which are looped byproducts formed during pre-mRNA splicing. DBR1’s catalytic activity is highly specific, and its function is essential for efficient RNA processing, as failure to debranch lariat RNAs causes their abnormal accumulation and disrupts gene expression regulation. Structurally, DBR1 requires cofactors such as Fe²⁺ and Zn²⁺ and interacts with regulatory partners like DRN1 and TTDN1 to enhance its debranching kinetics. The enzyme is predominantly nuclear and its debranching role is conserved from yeast to humans, with >70% of human genes containing introns whose turnover depends on DBR1. Deficiency of DBR1 leads to severe cellular consequences, such as embryonic lethality in animals and plants, and is implicated in human diseases including autosomal recessive brainstem viral-induced encephalitis, lethal congenital ichthyosis, and potentially neurodegenerative conditions like ALS.
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技术参数
- 靶点基因名
- DBR1
- 蛋白别名
- debranching RNA lariats 1; DBR1
- UniProt
- Q9UK59
- 物种
- Human
产品详情
- 靶点名称
- DBR1
- 蛋白名称
- debranching RNA lariats 1
- 蛋白别名
- Lariat debranching enzyme
- 靶点基因名
- DBR1
- UniProt
- Q9UK59
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Debranching RNA lariats 1 (DBR1) is an evolutionarily conserved protein that acts as the sole enzyme responsible for hydrolyzing the 2′–5′ phosphodiester bond in lariat RNAs, which are looped byproducts formed during pre-mRNA splicing. DBR1’s catalytic activity is highly specific, and its function is essential for efficient RNA processing, as failure to debranch lariat RNAs causes their abnormal accumulation and disrupts gene expression regulation. Structurally, DBR1 requires cofactors such as Fe²⁺ and Zn²⁺ and interacts with regulatory partners like DRN1 and TTDN1 to enhance its debranching kinetics. The enzyme is predominantly nuclear and its debranching role is conserved from yeast to humans, with >70% of human genes containing introns whose turnover depends on DBR1. Deficiency of DBR1 leads to severe cellular consequences, such as embryonic lethality in animals and plants, and is implicated in human diseases including autosomal recessive brainstem viral-induced encephalitis, lethal congenital ichthyosis, and potentially neurodegenerative conditions like ALS.