| Gene Symbol | NDUFS3 |
| Entrez Gene ID | 4722 |
| Full Name | NADH:ubiquinone oxidoreductase core subunit S3 |
| Synonyms | CI-30 |
| General protein information |
|
| Gene Type | protein-coding |
| Organism | Homo sapiens(human) |
| Genome | |
| Summary | This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.[provided by RefSeq, Apr 2009]. |
| Disorder MIM: | |
| Disorder Html: | Leigh syndrome due to mitochondrial complex I deficiency, 256000 (3); Mitochondrial complex I deficiency, 252010 (3) |
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NDUFS3 cDNA ORF clone, Homo sapiens(human)
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Gene
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Clones
gRNAs
The following NDUFS3 gene cDNA ORF clone sequences were retrieved from the NCBI Reference Sequence Database (RefSeq). These sequences represent the protein coding region of the NDUFS3 cDNA ORF which is encoded by the open reading frame (ORF) sequence. ORF sequences can be delivered in our standard vector, pcDNA3.1+/C-(K)DYK or the vector of your choice as an expression/transfection-ready ORF clone. Not the clone you want? Click here to find your clone.
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Reference Sequences (Refseq)
CloneID OHu19386 
Clone ID Related Accession (Same CDS sequence) NM_004551.2 , NM_004551.3 Accession Version NM_004551.2 Documents for ORF clone product in default vector Sequence Information ORF Nucleotide Sequence (Length: 795bp)
Protein sequence
SNPVector pcDNA3.1-C-(k)DYK or customized vector 
User ManualClone information Clone Map MSDSTag on pcDNA3.1+/C-(K)DYK C terminal DYKDDDDK tags ORF Insert Method CloneEZ™ Seamless cloning technology Insert Structure linear Update Date 1514217600000 Organism Homo sapiens(human) Product NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial precursor Comment Comment: REVIEWED REFSEQ: This record has been curated by NCBI staff. The reference sequence was derived from DA893277.1, BC000617.2 and AA477836.1. This sequence is a reference standard in the RefSeqGene project. On Apr 7, 2009 this sequence version replaced NM_004551.1. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Gene record to access additional publications. ##Evidence-Data-START## Transcript exon combination :: AK223255.1, SRR1163658.556262.1 [ECO:0000332] RNAseq introns :: single sample supports all introns SAMEA1965299, SAMEA1966682 [ECO:0000348] ##Evidence-Data-END## ##RefSeq-Attributes-START## gene product(s) localized to mito. :: reported by MitoCarta ##RefSeq-Attributes-END## COMPLETENESS: complete on the 3' end.
1
61
121
181
241
301
361
421
481
541
601
661
721
781ATGGCGGCGG CGGCGGTAGC CAGGCTGTGG TGGCGCGGGA TCTTGGGGGC CTCGGCGCTG
ACCAGGGGGA CTGGGCGACC CTCCGTTCTG TTGCTGCCGG TGAGGCGGGA GAGCGCCGGG
GCCGACACGC GCCCCACTGT CAGACCACGG AATGATGTGG CCCACAAGCA GCTCTCAGCT
TTTGGAGAGT ATGTGGCTGA AATCTTGCCC AAGTATGTCC AACAAGTTCA GGTGTCCTGC
TTCAATGAGT TAGAGGTCTG TATCCATCCT GATGGCGTCA TCCCAGTGCT GACTTTCCTC
AGGGATCACA CCAATGCACA GTTCAAATCT CTGGTTGACT TGACAGCAGT GGACGTCCCA
ACTCGGCAAA ACCGTTTTGA GATTGTCTAC AACCTGTTGT CTCTGCGCTT CAACTCACGG
ATCCGTGTGA AGACCTACAC AGATGAGCTG ACGCCCATTG AGTCTGCTGT CTCTGTGTTC
AAGGCAGCCA ACTGGTATGA AAGGGAGATC TGGGACATGT TTGGAGTCTT CTTTGCTAAC
CACCCTGATC TAAGAAGGAT CCTGACAGAT TATGGCTTCG AGGGACATCC TTTCCGGAAA
GACTTTCCTC TATCTGGCTA TGTTGAGTTA CGTTATGATG ATGAAGTGAA GCGGGTGGTG
GCAGAGCCGG TGGAGTTGGC CCAAGAGTTC CGCAAATTTG ACCTGAACAG CCCCTGGGAG
GCTTTCCCAG TCTATCGCCA ACCCCCGGAG AGTCTCAAGC TTGAAGCCGG AGACAAGAAG
CCTGATGCCA AGTAGThe stop codons will be deleted if pcDNA3.1+/C-(K)DYK vector is selected.
RefSeq NP_004542.1 CDS 83..877 Translation 
Target ORF information:
RefSeq Version NM_004551.2 Organism Homo sapiens(human) Definition Homo sapiens NADH:ubiquinone oxidoreductase core subunit S3 (NDUFS3), mRNA. Target ORF information:
Epitope DYKDDDDK Bacterial selection AMPR Mammalian selection NeoR Vector pcDNA3.1+/C-(K)DYK 
NM_004551.2
ORF Insert Sequence:
1
61
121
181
241
301
361
421
481
541
601
661
721
781ATGGCGGCGG CGGCGGTAGC CAGGCTGTGG TGGCGCGGGA TCTTGGGGGC CTCGGCGCTG
ACCAGGGGGA CTGGGCGACC CTCCGTTCTG TTGCTGCCGG TGAGGCGGGA GAGCGCCGGG
GCCGACACGC GCCCCACTGT CAGACCACGG AATGATGTGG CCCACAAGCA GCTCTCAGCT
TTTGGAGAGT ATGTGGCTGA AATCTTGCCC AAGTATGTCC AACAAGTTCA GGTGTCCTGC
TTCAATGAGT TAGAGGTCTG TATCCATCCT GATGGCGTCA TCCCAGTGCT GACTTTCCTC
AGGGATCACA CCAATGCACA GTTCAAATCT CTGGTTGACT TGACAGCAGT GGACGTCCCA
ACTCGGCAAA ACCGTTTTGA GATTGTCTAC AACCTGTTGT CTCTGCGCTT CAACTCACGG
ATCCGTGTGA AGACCTACAC AGATGAGCTG ACGCCCATTG AGTCTGCTGT CTCTGTGTTC
AAGGCAGCCA ACTGGTATGA AAGGGAGATC TGGGACATGT TTGGAGTCTT CTTTGCTAAC
CACCCTGATC TAAGAAGGAT CCTGACAGAT TATGGCTTCG AGGGACATCC TTTCCGGAAA
GACTTTCCTC TATCTGGCTA TGTTGAGTTA CGTTATGATG ATGAAGTGAA GCGGGTGGTG
GCAGAGCCGG TGGAGTTGGC CCAAGAGTTC CGCAAATTTG ACCTGAACAG CCCCTGGGAG
GCTTTCCCAG TCTATCGCCA ACCCCCGGAG AGTCTCAAGC TTGAAGCCGG AGACAAGAAG
CCTGATGCCA AGTAGThe stop codons will be deleted if pcDNA3.1+/C-(K)DYK vector is selected.
CloneID OHu19386 Clone ID Related Accession (Same CDS sequence) NM_004551.2 , NM_004551.3 Accession Version NM_004551.3 Latest version! Documents for ORF clone product in default vector Sequence Information ORF Nucleotide Sequence (Length: 795bp)
Protein sequence
SNPVector pcDNA3.1-C-(k)DYK or customized vector User ManualClone information Clone Map MSDSTag on pcDNA3.1+/C-(K)DYK C terminal DYKDDDDK tags ORF Insert Method CloneEZ™ Seamless cloning technology Insert Structure linear Update Date 1568995200000 Organism Homo sapiens(human) Product NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial precursor Comment Comment: REVIEWED REFSEQ: This record has been curated by NCBI staff. The reference sequence was derived from BC000617.2. This sequence is a reference standard in the RefSeqGene project. On Nov 23, 2018 this sequence version replaced NM_004551.2. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Gene record to access additional publications. ##Evidence-Data-START## Transcript exon combination :: BC000617.2, SRR1163655.444410.1 [ECO:0000332] RNAseq introns :: single sample supports all introns SAMEA1965299, SAMEA1966682 [ECO:0000348] ##Evidence-Data-END## ##RefSeq-Attributes-START## gene product(s) localized to mito. :: reported by MitoCarta MANE Ensembl match :: ENST00000263774.9/ ENSP00000263774.4 RefSeq Select criteria :: based on single protein-coding transcript ##RefSeq-Attributes-END## COMPLETENESS: full length.
1
61
121
181
241
301
361
421
481
541
601
661
721
781ATGGCGGCGG CGGCGGTAGC CAGGCTGTGG TGGCGCGGGA TCTTGGGGGC CTCGGCGCTG
ACCAGGGGGA CTGGGCGACC CTCCGTTCTG TTGCTGCCGG TGAGGCGGGA GAGCGCCGGG
GCCGACACGC GCCCCACTGT CAGACCACGG AATGATGTGG CCCACAAGCA GCTCTCAGCT
TTTGGAGAGT ATGTGGCTGA AATCTTGCCC AAGTATGTCC AACAAGTTCA GGTGTCCTGC
TTCAATGAGT TAGAGGTCTG TATCCATCCT GATGGCGTCA TCCCAGTGCT GACTTTCCTC
AGGGATCACA CCAATGCACA GTTCAAATCT CTGGTTGACT TGACAGCAGT GGACGTCCCA
ACTCGGCAAA ACCGTTTTGA GATTGTCTAC AACCTGTTGT CTCTGCGCTT CAACTCACGG
ATCCGTGTGA AGACCTACAC AGATGAGCTG ACGCCCATTG AGTCTGCTGT CTCTGTGTTC
AAGGCAGCCA ACTGGTATGA AAGGGAGATC TGGGACATGT TTGGAGTCTT CTTTGCTAAC
CACCCTGATC TAAGAAGGAT CCTGACAGAT TATGGCTTCG AGGGACATCC TTTCCGGAAA
GACTTTCCTC TATCTGGCTA TGTTGAGTTA CGTTATGATG ATGAAGTGAA GCGGGTGGTG
GCAGAGCCGG TGGAGTTGGC CCAAGAGTTC CGCAAATTTG ACCTGAACAG CCCCTGGGAG
GCTTTCCCAG TCTATCGCCA ACCCCCGGAG AGTCTCAAGC TTGAAGCCGG AGACAAGAAG
CCTGATGCCA AGTAGThe stop codons will be deleted if pcDNA3.1+/C-(K)DYK vector is selected.
RefSeq NP_004542.1 CDS 19..813 Translation
Target ORF information:
RefSeq Version NM_004551.3 Organism Homo sapiens(human) Definition Homo sapiens NADH:ubiquinone oxidoreductase core subunit S3 (NDUFS3), mRNA. Target ORF information:
Epitope DYKDDDDK Bacterial selection AMPR Mammalian selection NeoR Vector pcDNA3.1+/C-(K)DYK NM_004551.3
ORF Insert Sequence:
1
61
121
181
241
301
361
421
481
541
601
661
721
781ATGGCGGCGG CGGCGGTAGC CAGGCTGTGG TGGCGCGGGA TCTTGGGGGC CTCGGCGCTG
ACCAGGGGGA CTGGGCGACC CTCCGTTCTG TTGCTGCCGG TGAGGCGGGA GAGCGCCGGG
GCCGACACGC GCCCCACTGT CAGACCACGG AATGATGTGG CCCACAAGCA GCTCTCAGCT
TTTGGAGAGT ATGTGGCTGA AATCTTGCCC AAGTATGTCC AACAAGTTCA GGTGTCCTGC
TTCAATGAGT TAGAGGTCTG TATCCATCCT GATGGCGTCA TCCCAGTGCT GACTTTCCTC
AGGGATCACA CCAATGCACA GTTCAAATCT CTGGTTGACT TGACAGCAGT GGACGTCCCA
ACTCGGCAAA ACCGTTTTGA GATTGTCTAC AACCTGTTGT CTCTGCGCTT CAACTCACGG
ATCCGTGTGA AGACCTACAC AGATGAGCTG ACGCCCATTG AGTCTGCTGT CTCTGTGTTC
AAGGCAGCCA ACTGGTATGA AAGGGAGATC TGGGACATGT TTGGAGTCTT CTTTGCTAAC
CACCCTGATC TAAGAAGGAT CCTGACAGAT TATGGCTTCG AGGGACATCC TTTCCGGAAA
GACTTTCCTC TATCTGGCTA TGTTGAGTTA CGTTATGATG ATGAAGTGAA GCGGGTGGTG
GCAGAGCCGG TGGAGTTGGC CCAAGAGTTC CGCAAATTTG ACCTGAACAG CCCCTGGGAG
GCTTTCCCAG TCTATCGCCA ACCCCCGGAG AGTCTCAAGC TTGAAGCCGG AGACAAGAAG
CCTGATGCCA AGTAGThe stop codons will be deleted if pcDNA3.1+/C-(K)DYK vector is selected.
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PubMed
A Novel NDUFS3 mutation in a Chinese patient with severe Leigh syndrome.
Journal of human genetics63(12)1269-1272(2018 Dec)
Lou X,Shi H,Wen S,Li Y,Wei X,Xie J,Ma L,Yang Y,Fang H,Lyu J
Downregulation of DJ-1 Fails to Protect Mitochondrial Complex I Subunit NDUFS3 in the Testes and Contributes to the Asthenozoospermia.
Mediators of inflammation20186136075(2018)
Wang Y,Sun Y,Zhao X,Yuan R,Jiang H,Pu X
Using a quantitative quadruple immunofluorescent assay to diagnose isolated mitochondrial Complex I deficiency.
Scientific reports7(1)15676(2017 Nov)
Ahmed ST,Alston CL,Hopton S,He L,Hargreaves IP,Falkous G,Oláhová M,McFarland R,Turnbull DM,Rocha MC,Taylor RW
Altered monocyte expression and expansion of non-classical monocyte subset in IgA nephropathy patients.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association30(7)1122-232(2015 Jul)
Cox SN,Serino G,Sallustio F,Blasi A,Rossini M,Pesce F,Schena FP
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