m6A Regulator Information

General Information of the m6A Regulator (ID: REG00044)
Regulator Name Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC)
Synonyms
LRP130; 130 kDa leucine-rich protein; GP130; LRP 130
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Gene Name LRPPRC
Sequence
MAALLRSARWLLRAGAAPRLPLSLRLLPGGPGRLHAASYLPAARAGPVAGGLLSPARLYA
IAAKEKDIQEESTFSSRKISNQFDWALMRLDLSVRRTGRIPKKLLQKVFNDTCRSGGLGG
SHALLLLRSCGSLLPELKLEERTEFAHRIWDTLQKLGAVYDVSHYNALLKVYLQNEYKFS
PTDFLAKMEEANIQPNRVTYQRLIASYCNVGDIEGASKILGFMKTKDLPVTEAVFSALVT
GHARAGDMENAENILTVMRDAGIEPGPDTYLALLNAYAEKGDIDHVKQTLEKVEKSELHL
MDRDLLQIIFSFSKAGYPQYVSEILEKVTCERRYIPDAMNLILLLVTEKLEDVALQILLA
CPVSKEDGPSVFGSFFLQHCVTMNTPVEKLTDYCKKLKEVQMHSFPLQFTLHCALLANKT
DLAKALMKAVKEEGFPIRPHYFWPLLVGRRKEKNVQGIIEILKGMQELGVHPDQETYTDY
VIPCFDSVNSARAILQENGCLSDSDMFSQAGLRSEAANGNLDFVLSFLKSNTLPISLQSI
RSSLLLGFRRSMNINLWSEITELLYKDGRYCQEPRGPTEAVGYFLYNLIDSMSDSEVQAK
EEHLRQYFHQLEKMNVKIPENIYRGIRNLLESYHVPELIKDAHLLVESKNLDFQKTVQLT
SSELESTLETLKAENQPIRDVLKQLILVLCSEENMQKALELKAKYESDMVTGGYAALINL
CCRHDKVEDALNLKEEFDRLDSSAVLDTGKYVGLVRVLAKHGKLQDAINILKEMKEKDVL
IKDTTALSFFHMLNGAALRGEIETVKQLHEAIVTLGLAEPSTNISFPLVTVHLEKGDLST
ALEVAIDCYEKYKVLPRIHDVLCKLVEKGETDLIQKAMDFVSQEQGEMVMLYDLFFAFLQ
TGNYKEAKKIIETPGIRARSARLQWFCDRCVANNQVETLEKLVELTQKLFECDRDQMYYN
LLKLYKINGDWQRADAVWNKIQEENVIPREKTLRLLAEILREGNQEVPFDVPELWYEDEK
HSLNSSSASTTEPDFQKDILIACRLNQKKGAYDIFLNAKEQNIVFNAETYSNLIKLLMSE
DYFTQAMEVKAFAETHIKGFTLNDAANSRLIITQVRRDYLKEAVTTLKTVLDQQQTPSRL
AVTRVIQALAMKGDVENIEVVQKMLNGLEDSIGLSKMVFINNIALAQIKNNNIDAAIENI
ENMLTSENKVIEPQYFGLAYLFRKVIEEQLEPAVEKISIMAERLANQFAIYKPVTDFFLQ
LVDAGKVDDARALLQRCGAIAEQTPILLLFLLRNSRKQGKASTVKSVLELIPELNEKEEA
YNSLMKSYVSEKDVTSAKALYEHLTAKNTKLDDLFLKRYASLLKYAGEPVPFIEPPESFE
FYAQQLRKLRENSS
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Function
May play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus 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. Positively modulates nuclear export of mRNAs containing the EIF4E sensitivity element (4ESE) by binding simultaneously to both EIF4E and the 4ESE and acting as a platform for assembly for the RNA export complex. Also binds to exportin XPO1/CRM1 to engage the nuclear pore and traffic the bound mRNAs to the cytoplasm. May bind mature mRNA in the nucleus outer membrane. In mitochondria binds to poly(A) mRNA. Plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. May be involved in transcription regulation. Cooperates with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A to transcription factors. Seems to be involved in the transcription regulation of the multidrug-related genes MDR1 and MVP. Part of a nuclear factor that binds to the invMED1 element of MDR1 and MVP gene promoters. Binds single-stranded DNA (By similarity).
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Gene ID 10128
Uniprot ID
LPPRC_HUMAN
Regulator Type WRITER ERASER READER
Full List of Target Gene(s) of This m6A Regulator and Corresponding Disease/Drug Response(s)
LRPPRC can regulate the m6A methylation of following target genes, and result in corresponding disease/drug response(s). You can browse corresponding disease or drug response(s) resulted from the regulation of certain target gene.
Browse Target Gene related Disease
Programmed cell death 1 ligand 1 (CD274/PD-L1)
 Target Gene 
Liver cancer [ICD-11: 2C12]
In total 1 item(s) under this disease
 Disease Info 
Experiment 1 Reporting the m6A-centered Disease Response of This Target Gene [1]
Responsed Disease Liver hepatocellular carcinoma [ICD-11: 2C12.02]
Target Regulation Up regulation
In-vitro Model
 Hep-G2 Hepatoblastoma Homo sapiens CVCL_0027
Hep 3B2.1-7 Childhood hepatocellular carcinoma Homo sapiens CVCL_0326
In-vivo Model Male BALB/c nude mice (18-20 g weight, 6-week-old) were acquired from Beijing Vital River Laboratory Animal Technology Co., Ltd. (China). Xenograft tumors were produced through subcutaneously injecting 2×105 control or sh-LRPPRC-transfected HepG2 cells under the arm. Tumor growth was measured by use of slide caliper every 3 days. Tumor volume was computed utilizing the formula 1/2 × (length × width2).
Click to View Potential Drug Response of This Regulator
Full List of Crosstalk(s) between m6A Modification and Epigenetic Regulation Related to This Regulator
RNA modification
m6A Target: Zinc finger protein SNAI1 (SNAI1)
 Target Gene 
In total 1 item(s) under this m6A target
Crosstalk ID: M6ACROT00487
 Crosstalk Info 
Epigenetic Regulator Fat mass and obesity-associated protein (FTO)
Regulated Target hsa-mir-22
Crosstalk relationship m6Am → m6A
m6A Target: Glucose transporter type 1 (GLUT1)
 Target Gene 
In total 1 item(s) under this m6A target
Crosstalk ID: M6ACROT00492
 Crosstalk Info 
Epigenetic Regulator Fat mass and obesity-associated protein (FTO)
Regulated Target hsa-mir-22
Crosstalk relationship m6Am → m6A
m6A Target: Differentiation antagonizing non-protein coding RNA (DANCR)
 Target Gene 
In total 3 item(s) under this m6A target
Crosstalk ID: M6ACROT00558
 Crosstalk Info 
Epigenetic Regulator Interferon-inducible protein 4 (ADAR1)
Regulated Target Transcription factor SOX-2 (SOX2)
Crosstalk relationship m6A → A-to-I
Crosstalk ID: M6ACROT00560
 Crosstalk Info 
Epigenetic Regulator Fat mass and obesity-associated protein (FTO)
Regulated Target Transcription factor SOX-2 (SOX2)
Crosstalk relationship m6A → m6Am
Crosstalk ID: M6ACROT00562
 Crosstalk Info 
Epigenetic Regulator Methyltransferase-like protein 1 (METTL1)
Regulated Target Transcription factor SOX-2 (SOX2)
Crosstalk relationship m6A → m7G
Non-coding RNA
m6A Target: Differentiation antagonizing non-protein coding RNA (DANCR)
 Target Gene 
In total 3 item(s) under this m6A target
Crosstalk ID: M6ACROT05958
 Crosstalk Info 
Epigenetic Regulator Differentiation antagonizing non-protein coding RNA (DANCR)
Regulated Target Interleukin-11 (IL11)
Crosstalk relationship m6A → ncRNA
Disease Bladder cancer
 Disease Info 
Drug BP-1-102
 Drug Info 
Crosstalk ID: M6ACROT05959
 Crosstalk Info 
Epigenetic Regulator Differentiation antagonizing non-protein coding RNA (DANCR)
Regulated Target G1/S-specific cyclin-D1 (CCND1)
Crosstalk relationship m6A → ncRNA
Disease Bladder cancer
 Disease Info 
Drug BP-1-102
 Drug Info 
Crosstalk ID: M6ACROT05960
 Crosstalk Info 
Epigenetic Regulator Differentiation antagonizing non-protein coding RNA (DANCR)
Regulated Target Urokinase-type plasminogen activator (PLAU)
Crosstalk relationship m6A → ncRNA
Disease Bladder cancer
 Disease Info 
Drug BP-1-102
 Drug Info 
References
Ref 1 A phase 1 open-label trial shows that smad7 antisense oligonucleotide (GED0301) does not increase the risk of small bowel strictures in Crohn's disease. Aliment Pharmacol Ther. 2012 Nov;36(9):850-7. doi: 10.1111/apt.12051.