KEGG   Homo sapiens (human): 10128
Entry
10128             CDS       T01001                                 
Symbol
LRPPRC, CLONE-23970, GP130, LRP130, LSFC, MC4DN5
Name
(RefSeq) leucine rich pentatricopeptide repeat containing
  KO
K17964  leucine-rich PPR motif-containing protein, mitochondrial
Organism
hsa  Homo sapiens (human)
Disease
H01354  Leigh syndrome
H01368  Cytochrome c oxidase (COX) deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis [BR:hsa03029]
    10128 (LRPPRC)
Mitochondrial biogenesis [BR:hsa03029]
 Mitochondrial quality control factors
  Mitochondrial respiratory chain complex assembly factors
   Complex-IV assembly factors
    10128 (LRPPRC)
SSDB
Motif
Pfam: PPR_2 PPR PPR_3 PPR_long PPR_1 TPR_19 MA3 TPR_MalT WipA_N
Other DBs
NCBI-GeneID: 10128
NCBI-ProteinID: NP_573566
OMIM: 607544
HGNC: 15714
Ensembl: ENSG00000138095
UniProt: P42704 E5KNY5
Structure
Position
2:complement(43886224..43996265)
AA seq 1394 aa
MAALLRSARWLLRAGAAPRLPLSLRLLPGGPGRLHAASYLPAARAGPVAGGLLSPARLYA
IAAKEKDIQEESTFSSRKISNQFDWALMRLDLSVRRTGRIPKKLLQKVFNDTCRSGGLGG
SHALLLLRSCGSLLPELKLEERTEFAHRIWDTLQKLGAVYDVSHYNALLKVYLQNEYKFS
PTDFLAKMEEANIQPNRVTYQRLIASYCNVGDIEGASKILGFMKTKDLPVTEAVFSALVT
GHARAGDMENAENILTVMRDAGIEPGPDTYLALLNAYAEKGDIDHVKQTLEKVEKSELHL
MDRDLLQIIFSFSKAGYPQYVSEILEKVTCERRYIPDAMNLILLLVTEKLEDVALQILLA
CPVSKEDGPSVFGSFFLQHCVTMNTPVEKLTDYCKKLKEVQMHSFPLQFTLHCALLANKT
DLAKALMKAVKEEGFPIRPHYFWPLLVGRRKEKNVQGIIEILKGMQELGVHPDQETYTDY
VIPCFDSVNSARAILQENGCLSDSDMFSQAGLRSEAANGNLDFVLSFLKSNTLPISLQSI
RSSLLLGFRRSMNINLWSEITELLYKDGRYCQEPRGPTEAVGYFLYNLIDSMSDSEVQAK
EEHLRQYFHQLEKMNVKIPENIYRGIRNLLESYHVPELIKDAHLLVESKNLDFQKTVQLT
SSELESTLETLKAENQPIRDVLKQLILVLCSEENMQKALELKAKYESDMVTGGYAALINL
CCRHDKVEDALNLKEEFDRLDSSAVLDTGKYVGLVRVLAKHGKLQDAINILKEMKEKDVL
IKDTTALSFFHMLNGAALRGEIETVKQLHEAIVTLGLAEPSTNISFPLVTVHLEKGDLST
ALEVAIDCYEKYKVLPRIHDVLCKLVEKGETDLIQKAMDFVSQEQGEMVMLYDLFFAFLQ
TGNYKEAKKIIETPGIRARSARLQWFCDRCVANNQVETLEKLVELTQKLFECDRDQMYYN
LLKLYKINGDWQRADAVWNKIQEENVIPREKTLRLLAEILREGNQEVPFDVPELWYEDEK
HSLNSSSASTTEPDFQKDILIACRLNQKKGAYDIFLNAKEQNIVFNAETYSNLIKLLMSE
DYFTQAMEVKAFAETHIKGFTLNDAANSRLIITQVRRDYLKEAVTTLKTVLDQQQTPSRL
AVTRVIQALAMKGDVENIEVVQKMLNGLEDSIGLSKMVFINNIALAQIKNNNIDAAIENI
ENMLTSENKVIEPQYFGLAYLFRKVIEEQLEPAVEKISIMAERLANQFAIYKPVTDFFLQ
LVDAGKVDDARALLQRCGAIAEQTPILLLFLLRNSRKQGKASTVKSVLELIPELNEKEEA
YNSLMKSYVSEKDVTSAKALYEHLTAKNTKLDDLFLKRYASLLKYAGEPVPFIEPPESFE
FYAQQLRKLRENSS
NT seq 4185 nt   +upstreamnt  +downstreamnt
atggcagccctgctgagatccgcgcgttggttgctgcgtgccggggcggccccgcgcctc
ccgctctccctgcgcctcctccctggcggcccgggccggctgcatgccgcctcctatctg
cccgccgctcgcgccgggcccgtggccggaggactactgagcccagccaggctgtatgcc
attgctgccaaagaaaaagatattcaagaggagtccactttttcttctaggaagatttcc
aatcagtttgattgggctctaatgagactagatctttctgttcgaagaactggccgcatt
ccaaagaagcttctacaaaaagtttttaatgatacctgccgctcaggtggcctaggtggt
agtcatgccttgcttctactacgtagttgtggttctctcttgcctgaactaaagcttgaa
gagagaacagaatttgctcataggatatgggacacacttcagaaattaggtgctgtgtat
gatgtgagtcactataatgctttacttaaagtctatcttcaaaatgaatataaattctca
ccaactgatttcctggcaaaaatggaggaagcaaacattcaaccaaatcgagtgacatac
cagagattgattgcttcttattgtaatgtaggagatattgaaggtgccagcaagattctt
ggatttatgaaaactaaggatctcccagttacagaggcagtattcagtgcccttgtgaca
gggcatgccagagctggtgatatggagaatgcagaaaacattctcacagtgatgagagat
gccggaattgagcctggtccagacacatacctcgcattattgaatgcatatgctgagaag
ggcgacattgaccatgttaagcagactctggagaaggtggagaagtccgagcttcacctt
atggaccgtgatttactgcaaattatttttagcttcagtaaagctgggtatcctcagtat
gtctcagaaattttggaaaaagttacatgtgaaagaagatatattccagatgcaatgaac
ctcattttacttttagtcactgaaaaattggaagatgtagcgttgcaaattttactagca
tgccccgtatcaaaggaagatggcccaagtgtctttggcagtttctttttacaacactgt
gtgactatgaatacgcctgtggagaagctaacagactactgtaagaagttaaaggaagtc
cagatgcactcctttcctctgcagttcaccctccattgtgctttactcgccaataaaact
gatttggcaaaagccttaatgaaggctgtgaaggaggaaggttttcctatcagacctcac
tatttctggccattgctagttggacgtcggaaggaaaaaaatgttcaaggtataattgaa
atcctcaaaggaatgcaagaattgggagtacatcctgatcaggaaacatatacagattat
gtgattccatgctttgatagtgtaaactcagcacgagccattttgcaggaaaatggatgt
ctgtctgatagtgatatgttttctcaagctggattgagaagtgaagcagcaaatgggaac
ttagactttgtattatcatttttgaaatcaaatacattgcccatctcgctgcagtctata
agaagtagcctactgctaggcttcaggaggtctatgaatataaatctttggagcgagata
acagaattgttgtacaaggatggacgttattgccaggagcctcgaggaccgacggaagct
gttggctattttctttataacttgattgacagcatgagtgactcagaggtacaggccaag
gaggagcatttgagacaatacttccatcagctggagaagatgaatgtaaaaattcctgaa
aatatctacagaggcattcgtaatctcctggaaagctaccatgttcctgaattgattaag
gatgctcacttgttggttgagagtaagaatttagactttcaaaaaactgtgcaacttaca
tcatctgaattggagtccacacttgaaacactaaaagctgaaaatcaacctataagagat
gtcctaaagcaactcatattagtgctttgttcagaagagaatatgcaaaaagcccttgaa
ttgaaagcaaaatatgaatccgacatggttactggtggctatgcagctttaataaattta
tgctgtcgacatgataaagtagaagatgccttgaacttgaaagaagaatttgaccgctta
gattcatctgctgtccttgacaccggcaagtatgtaggccttgtaagagtattggcaaag
catggcaagctccaagatgctattaacattctgaaggagatgaaagagaaggatgttctt
atcaaagatacaacagccttgtcctttttccacatgctaaatggcgcagctttaagaggt
gaaattgaaacagtaaaacagttgcatgaagccatcgtgactctagggttagcagaacca
tccaccaacataagtttcccattggtcactgtacacttggaaaagggcgacctatctact
gctcttgaggtcgccattgactgctatgaaaagtataaagtattaccaaggattcatgat
gtcttgtgtaaactggtagagaaaggcgagactgatctaattcagaaagcaatggacttt
gtgagccaagaacaaggtgaaatggtgatgctctatgatctcttctttgccttcctacaa
acaggaaattacaaagaggccaagaagatcattgagactccagggattagagctcgatct
gcaaggcttcagtggttttgtgacagatgtgttgcaaataatcaggttgaaactctggaa
aaattagtggagctgacacagaagctatttgaatgtgatagagaccagatgtactacaat
ctgctaaaactgtataaaataaacggtgactggcaaagagctgatgcagtctggaataaa
atccaagaagaaaatgttattcctcgtgaaaagacattaagattattagcagaaatcctt
agagagggtaaccaggaagttccgtttgacgtacctgagttgtggtatgaagatgaaaaa
cattccctgaattcttcgtcagcctcaaccacagaacctgatttccagaaagatatattg
attgcctgccgattgaaccaaaaaaaaggggcatatgatattttcctgaatgcaaaagag
caaaacattgtgtttaatgctgaaacctacagcaatctcattaaattactgatgtcagaa
gattattttacacaagcaatggaagtgaaagcattcgcggagacccacatcaagggcttc
acactgaacgatgctgccaacagccgcctcatcataacgcaagttaggcgggattatttg
aaagaggctgtgacaacactgaaaacagtattggatcagcagcagaccccttctaggtta
gcagtgacccgtgtcatccaggcattggccatgaagggtgatgttgaaaacatagaagta
gttcagaagatgttaaatggactcgaagactccattggactttcaaaaatggttttcatc
aataacattgctttggctcaaataaagaataataacatagatgccgcaatagaaaacatt
gaaaatatgcttacttcagagaataaagtcattgaaccccaatacttcggcttggcatac
ttattcagaaaagtaatagaggagcagttggaaccagcagttgaaaagataagcatcatg
gcggagagattggccaatcagtttgcaatttataaacctgtcactgattttttccttcaa
cttgtggatgcaggcaaggtggatgatgccagagctctcctacagagatgtggtgcaatt
gctgaacaaaccccgattttgttgttgttcctccttaggaattctaggaaacaaggaaag
gcatcaactgtgaaatctgtgttagaattgattcctgaattaaatgaaaaggaagaagca
tacaattccctcatgaaaagctatgtctcagagaaagatgtcacatctgctaaagcactg
tatgaacatttgactgcaaagaatacaaaattggatgatctgtttctaaagcgttacgca
tctttgctgaagtatgctggagagcctgtccctttcattgaaccccctgaaagctttgaa
ttttatgcacagcagctaagaaaattgagggaaaactcttcttga

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Ontology (2)   
   KEGG BRITE (2)   
Disease (4)   
   KEGG DISEASE (2)   
   OMIM (2)   
Genome (1)   
   KEGG GENOME (1)   
Gene (28)   
   KEGG ORTHOLOGY (1)   
   RefGene (14)   
   NCBI-PROTEINID (1)   
   NCBI-Gene (1)   
   HGNC (1)   
   Ensembl (1)   
   RIKEN BRC-DNA (8)   
   OC (1)   
Protein sequence (10)   
   UniProt (2)   
   SWISS-PROT (1)   
   RefSeq(pep) (7)   
DNA sequence (37)   
   RefSeq(nuc) (9)   
   GenBank (14)   
   EMBL (14)   
3D Structure (1)   
   PDB (1)   
Protein domain (9)   
   Pfam (9)   
All databases (92)   

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