Tag/Conjugate
Unconjugated
Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. Information available upon request.
Antigen Description
This gene encodes a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). The precise role of this protein is unknown but studies suggest it may play a role in cytoskeletal organization, vesicular transport, or in transcriptional regulation of both nuclear and mitochondrial genes. The protein localizes primarily to mitochondria and is predicted to have an N-terminal mitochondrial targeting sequence. Mutations in this gene are associated with the French-Canadian type of Leigh syndrome. [provided by RefSeq, Mar 2012]
Function
RNA binding; NOT actin filament binding; beta-tubulin binding; microtubule binding; poly(A) RNA binding; protein binding; single-stranded DNA binding;
Synonyms
LRPPRC; leucine-rich pentatricopeptide repeat containing; LSFC; GP130; LRP130; CLONE-23970; leucine-rich PPR motif-containing protein, mitochondrial; LRP 130; 130 kDa leucine-rich protein; leucine-rich PPR-motif containing; mitochondrial leucine-rich PPR motif-containing protein;
Citations
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Zou, J; Yue, F; et al. Mitochondrion-associated protein LRPPRC suppresses the initiation of basal levels of autophagy via enhancing Bcl-2 stability. BIOCHEMICAL JOURNAL 454:447-457(2013).
Arun, V; Wiley, JC; et al. A novel neurofibromin (NF1) interaction with the leucine-rich pentatricopeptide repeat motif-containing protein links neurofibromatosis type 1 and the french canadian variant of leigh's syndrome in a common molecular complex. JOURNAL OF NEUROSCIENCE RESEARCH 91:494-505(2013).
COA
Certificate of Analysis
