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LRPPRC encodes a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). Additionally we are shipping LRPPRC Kits (6) and LRPPRC Proteins (3) and many more products for this protein.
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Human Polyclonal LRPPRC Primary Antibody for ICC, IF - ABIN442703
Kasashima, Sumitani, Endo: Human mitochondrial transcription factor A is required for the segregation of mitochondrial DNA in cultured cells. in Experimental cell research 2010
LRPPRC knock-down in mammalian cells leads to an imbalance between mitochondria-encoded and nuclear-encoded subunits of complex IV
study identifies LRPPRC as an important disease-causing gene in an early-onset, multisystem and neurological mitochondrial disease.
LRPPRC levels were reduced in muscle cells and undetectable in liver from French Canadian Leigh Syndrome patients.
LRPPRC is a transcription factor related to ABCB1 expression and highlight the importance of epigenetic regulation in CML resistance.
Downregulation of LRPPRC expression resulted in the reduced expression of Bcl-2 (show BCL2 Antibodies), upregulation of Bax (show BAX Antibodies), and cleaved caspase-9 (show CASP9 Antibodies) and caspase-3 (show CASP3 Antibodies). This induces apoptosis through the mitochondria-mediated pathway in PCa (show FLVCR1 Antibodies) cells.
LRPPRC functions as a checkpoint protein that prevents mitochondria from autophagy degradation and impact tumorigenesis.
Tetherin (show BST2 Antibodies) binds with the mitochondrion-associated autophagy suppressor LRPPRC and prohibits its association with the autophagy initiation complex.
LRPPRC overexpression is associated with gastric cancer.
Data indicate that C14C10.4/MMA-1 Is the Structural and functional homolog of mammalian LRPPRC.
LRPPRC therefore acts to suppress the initiation of basal levels of autophagy to clean up dysfunctional mitochondria and other cellular debris during the normal cell cycle.
both LRPPRC and SLIRP (show SLIRP Antibodies) are required for maintaining mt-mRNA steady-state levels independent of their roles in stabilizing the other partner of the LRPPRC-SLIRP (show SLIRP Antibodies) complex.
Ectopic expression of LRPPRC in the mouse liver completely interdicted non-alcoholic fatty liver disease, including inflammation.
LRPPRC deficiency in Leigh syndrome causes an ATP synthase deficiency.
LRPPRC does not directly regulate mtDNA transcription but rather acts as a post-transcriptional regulator of mammalian mtDNA expression.
Heart-specific Lrpprc disruption causes mitochondrial cardiomyopathy with drastic reduction in steady-state mitochondrial mRNA levels. LRPPRC forms an RNA-dependent protein complex needed to maintain a mitochondrial pool of non-translated mRNAs.
These data strongly suggest that LRPPRC is involved in the maturation of cytochrome c (show CYCS Antibodies) oxidase (COX (show CPOX Antibodies)), and is involved in stabilizing of mitochondrial mRNAs encoding COX (show CPOX Antibodies) transcripts.
LRP130 protein remodels mitochondria and stimulates fatty acid oxidation.
Our results demonstrate that LIF (show LIF Antibodies) signaling via the gp130/JAK (show JAK3 Antibodies)/STAT3 (show STAT3 Antibodies) pathway is required for the initiation of the astrogliosis-like reaction of retinal Muller cells after optic nerve injury.
LRP130 may function in stabilizing minisatellite DNA sequences.
These data link LRP130 and PGC-1alpha (show PPARGC1A Antibodies) to defective hepatic energy homeostasis in Leigh syndrome French Canadian variant , and reveal a novel regulatory mechanism of glucose homeostasis.
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.
130 kDa leucine-rich protein
, LRP 130
, leucine-rich PPR motif-containing protein, mitochondrial
, leucine-rich PPR-motif containing
, mitochondrial leucine-rich PPR motif-containing protein
, leucine rich protein 157
, leucine-rich protein 157
, leucine rich protein LRP130