基因名：
LRPPRC
产品别名：
CLONE-23970； GP130； LRP130； LSFC； MC4DN5； LRPPRC； leucine rich pentatricopeptide repeat containing； leucine rich pentatricopeptide repeat containing； leucine-rich PPR motif-containing protein, mitochondrial； 130 kDa leucine-rich protein； LRP 130； leucine-rich PPR-motif containing； mitochondrial leucine-rich PPR motif-containing protein； 富含亮氨酸蛋白LRP130；
背景信息：
Leucine-rich protein (LRP130) is a cytoplasmic mRNA-binding protein likely to be involved in the processing of mitochondrial DNA transcripts. Defects in the LRPPRC gene that encodes LRP130 result in the French-Canadian type of Leigh syndrome, a severe neurological disorder characterized by lesions in the subcortical region of the brain. LRP130 also interacts with the low-affinity receptor for leukemia inhibitory factor to produce an intracelluar signal cascade.
