Overview of LRPPRC molecular target information
Overview of LRPPRC molecular targets
Glycoprotein 130 (also known as gp130, IL6ST, IL6-beta, or CD130) is a signal transduction receptor element shared by the functional receptor complex of the IL-6 family of cytokines and belongs to the class I cytokine receptor family. The protein encoded by this gene is a signal transducer shared by multiple cytokines, including interleukin 6 (IL6), ciliary neurotrophic factor (CNTF), leukemia inhibitory factor (LIF), and tumor inhibitory factor M (OSM), and the activation of this protein depends on the binding of cytokines to their receptors. IL-6 or IL-11 binds to membrane-anchored or soluble IL-6r or IL-11r, respectively, initiating the binding of IL-6r (IL-11r) to gp130. For other IL-6 family cytokines, such as LIF and OSM, signal transduction is similarly activated by heterodimers of gp130 with LIF R or OSM R. Gene knockout studies in mice have shown that the gene encoding this protein plays a key role in regulating cardiomyocyte apoptosis.
Human LRPPRC Molecular Target Information
Molecular name: 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
Gene sequence:NCBI_Gene: 10128
Protein sequence: UniProtKB: P42704
Human LRPPRC target molecular function (prediction)
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 molecular target information
Molecular name: Lrpprc, leucine-rich PPR-motif containing
Alias:
- 3110001K13Rik
- C76645
- expressed sequence C76645
- Lrp130
- RIKEN cDNA 3110001K13 gene
Gene sequence:NCBI_Gene: 72416
Protein sequence:
Mouse Lrpprc target molecular function (prediction)
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 molecular target information
Molecular name: 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
Gene sequence:NCBI_Gene: 313867
Protein sequence:
Rat Lrpprc target molecular function (prediction)
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).