LRPPRC 靶点信息

LRPPRC 分子靶点信息概述

  • 分子名：LRPPRC, leucine rich pentatricopeptide repeat containing
  • 基因家族：Mitochondrial respiratory chain complex assembly factors; Pentatricopeptide repeat containing
  • 别名：GP130; LRP130
  • 曾用名：LSFC
  • 全称：Leigh syndrome, French-Canadian type (cytochrome oxidase deficiency)

LRPPRC 分子靶点综述

糖蛋白130(也称为gp130、IL6ST、IL6-beta或CD130)是细胞因子IL-6家族功能受体复合物所共有的信号转导受体元件，属于I类细胞因子受体家族。该基因编码的蛋白是多种细胞因子共有的信号转导子，包括白细胞介素6(IL6)、睫状神经营养因子(CNTF)、白血病抑制因子(LIF)和肿瘤抑制素M(OSM)，这种蛋白的激活依赖于细胞因子与其受体的结合。IL-6或IL-11分别与膜锚定或可溶性IL-6r或IL-11r结合，启动IL-6r(IL-11r)与gp130的结合。对于其他IL-6家族细胞因子，如LIF和OSM，信号转导同样由gp130与LIF R或OSM R的异二聚体激活。小鼠的基因敲除研究表明，编码这种蛋白的基因在调节心肌细胞凋亡中起着关键作用。

LRPPRC, leucine rich pentatricopeptide repeat containing 分子靶点种属信息

人 Human小鼠 Mouse大鼠 Rat

人 Human LRPPRC 分子靶点信息

  • 分子名：LRPPRC, leucine rich pentatricopeptide repeat containing
  • 别称：
    • 130 kDa leucine-rich protein
    • CLONE-23970
    • GP130
    • leucine-rich pentatricopeptide repeat containing
    • leucine-rich PPR motif-containing protein, mitochondrial
    • leucine-rich PPR-motif containing
    • LRP 130
    • LRP130
    • LSFC
    • MC4DN5
    • mitochondrial leucine-rich PPR motif-containing protein
  • 基因序列：NCBI_Gene: 10128
  • 蛋白序列：UniProtKB: P42704

人 Human LRPPRC靶点分子功能（预测）

Enables beta-tubulin binding activity and ubiquitin protein ligase binding activity. Predicted to be involved in mitochondrion transport along microtubule. Predicted to act upstream of or within negative regulation of mitochondrial RNA catabolic process and regulation of mitochondrial translation. Located in several cellular components, including condensed nuclear chromosome; mitochondrial nucleoid; and perinuclear region of cytoplasm. Colocalizes with mitochondrion. Implicated in French Canadian Leigh disease and Leigh disease.

小鼠 Mouse Lrpprc 分子靶点信息

小鼠 Mouse Lrpprc 靶点分子功能（预测）

Enables RNA binding activity and single-stranded DNA binding activity. Acts upstream of or within negative regulation of mitochondrial RNA catabolic process and regulation of mitochondrial translation. Located in mitochondrion and nucleus. Part of ribonucleoprotein complex. Is expressed in several structures, including brain; genitourinary system; heart; liver; and skeletal muscle. Human ortholog(s) of this gene implicated in French Canadian Leigh disease and Leigh disease. Orthologous to human LRPPRC (leucine rich pentatricopeptide repeat containing).

大鼠 Rat Lrpprc 分子靶点信息

  • 分子名：Lrpprc, leucine-rich pentatricopeptide repeat containing
  • 别称：
    • 130 kDa leucine-rich protein
    • leucine rich protein 157
    • leucine-rich PPR motif-containing protein, mitochondrial
    • leucine-rich PPR-motif containing
    • leucine-rich protein 157
    • LOC313867
    • LRP 130
    • Lrp157
    • rLRP157
  • 基因序列：NCBI_Gene: 313867
  • 蛋白序列：

大鼠 Rat Lrpprc 靶点分子功能（预测）

Predicted to enable beta-tubulin binding activity; nucleic acid binding activity; and ubiquitin protein ligase binding activity. Predicted to be involved in mRNA transport. Predicted to act upstream of or within negative regulation of mitochondrial RNA catabolic process and regulation of mitochondrial translation. Predicted to be located in several cellular components, including condensed nuclear chromosome; mitochondrial nucleoid; and perinuclear region of cytoplasm. Predicted to be part of ribonucleoprotein complex. Predicted to colocalize with mitochondrion. Human ortholog(s) of this gene implicated in French Canadian Leigh disease and Leigh disease. Orthologous to human LRPPRC (leucine rich pentatricopeptide repeat containing).

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