Anti-LRP130/LRPPRC Polyclonal Antibody is a Rabbit antibody targeting LRP130/LRPPRC. Anti-LRP130/LRPPRC Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF.

IgG

Rat (predicted:Human,Mouse,Chicken,Horse,Rabbit)

IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500

Polyclonal

KLH conjugated synthetic peptide: human LRP130

Human

LRPPRC

Leucine-rich PPR motif-containing protein, mitochondrial

LPPRC is thought to play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus, LPPRC binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation, which are possibly associated with nuclear mRNA export. In mitochondria, LPPRC binds to poly(A) mRNA. LPPRC may play a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. Defects in LRPPRC are the cause of Leigh syndrome French-Canadian type (LSFC). Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver.