HGNC ID: 15714
Target Abstract:
Glycoprotein 130 (also known as gp130, IL6ST, IL6-beta, or CD130) is a signaling receptor element shared by the cytokine IL-6 family of functional receptor complexes and belongs to the class I cytokine receptor family. The protein encoded by this gene is a signal transducer shared by several cytokines, including interleukin 6 (IL6), ciliary neurotrophic factor (CNTF), leukemia inhibitory factor (LIF), and oncosuppressor M (OSM), and the activation of this protein is dependent on the binding of the cytokine to its receptors.IL-6 or IL-11 binds to membrane-anchored or soluble IL-6r or IL- respectively 11r binding, which initiates the binding of IL-6r (IL-11r) to gp130. For other IL-6 family cytokines, such as LIF and OSM, signaling is similarly activated by the heterodimerization of gp130 with LIF R or OSM R. Knockout studies in mice have shown that the gene encoding this protein plays a key role in the regulation of cardiomyocyte apoptosis.
LRPPRC Target Infomation Overview
- Target Symbol: LRPPRC, leucine rich pentatricopeptide repeat containing
- Gene Groups: Mitochondrial respiratory chain complex assembly factors; Pentatricopeptide repeat containing
- Alias: GP130; LRP130
- Previous Names: LSFC
- Alias Names: Leigh syndrome, French-Canadian type (cytochrome oxidase deficiency)
LRPPRC, leucine rich pentatricopeptide repeat containing Target Infomation by Species
- Human
- Mouse
- Rat
Human LRPPRC Target Information
- Target Symbol: LRPPRC, leucine rich pentatricopeptide repeat containing
- Alias:
- 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
- NCBI_Gene: 10128
- UniProtKB: P42704
Human LRPPRC Predicted Functions
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.
Mouse Lrpprc Target Information
- Target Symbol: Lrpprc, leucine-rich PPR-motif containing
- Alias:
- 3110001K13Rik
- C76645
- expressed sequence C76645
- Lrp130
- RIKEN cDNA 3110001K13 gene
- NCBI_Gene: 72416
Mouse Lrpprc Predicted Functions
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).
Rat Lrpprc Target Information
- Target Symbol: Lrpprc, leucine-rich pentatricopeptide repeat containing
- Alias:
- 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
- NCBI_Gene: 313867
Rat Lrpprc Predicted Functions
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).
