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NDUFS3 - NADH:ubiquinone oxidoreductase core subunit S3 Gene

Also Known as CI-30; MC1DN8

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 4722

About NDUFS3

Cytogenetic location: 11p11.2 Genomic coordinates (GRCh38): 11:47,579,074-47,584,562 (from NCBI)

This gene has 15 transcripts (splice variants), 1 gene allele, 214 orthologues and is associated with 3 phenotypes. Ubiquitous expression in heart (RPKM 45.6), duodenum (RPKM 44.2) and 25 other tissues.

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]

NDUFS3 Products (1)

mRNA	Protein	Name
NM_004551.3	NP_004542.1	NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
contributes to NADH dehydrogenase (ubiquinone) activity	IMP IMP: Inferred from mutant phenotype	30140060	GOA
enables NADH dehydrogenase (ubiquinone) activity	IMP IMP: Inferred from mutant phenotype	14729820	GOA
enables NADH dehydrogenase activity	IMP IMP: Inferred from mutant phenotype	11112787	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	15250827	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in mitochondrial electron transport, NADH to ubiquinone	IMP IMP: Inferred from mutant phenotype	14729820	GOA
involved in mitochondrial respiratory chain complex I assembly	IMP IMP: Inferred from mutant phenotype	30140060	GOA
involved in reactive oxygen species metabolic process	IMP IMP: Inferred from mutant phenotype	16826196	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in mitochondrial inner membrane	IDA IDA: Inferred from direct assay	18826940	GOA
located in mitochondrial membrane	IDA IDA: Inferred from direct assay	17209039	GOA
part of respiratory chain complex I	IDA IDA: Inferred from direct assay	12611891	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

NDUFS3 Protein Structure

Complex1_30kDa

0
100
200
264 a.a.

Protein Preferred Names	Protein Names
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial	CI-30kD

NDUFS3 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	Anti Bait CoIP	15250827
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	Anti Tag CoIP	24344204
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	BN-PAGE	19688755
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	Anti Tag CoIP	33961781
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	Anti Tag CoIP	27499296
Intra	NDUFS3	O75489	NDUFS2	Homo sapiens	O75306	Anti Tag CoIP	28514442
Intra	NDUFS3	O75489	NDUFA8	Homo sapiens	P51970	BN-PAGE	21310150
Intra	NDUFS3	O75489	NDUFA8	Homo sapiens	P51970	Anti Tag CoIP	27499296
Intra	NDUFS3	O75489	TMEM11	Homo sapiens	P17152	Validated Y2H	32296183
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Y2H Array	32296183
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Anti Tag CoIP	24344204
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Anti Tag CoIP	27499296
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Validated Y2H	32296183
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Y2H Prey Pooling	32296183
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Anti Tag CoIP	33961781
Intra	NDUFS3	O75489	NDUFA5	Homo sapiens	Q16718	Anti Tag CoIP	28514442

Cross: Cross-species interaction Intra: Intraspecies interaction

NDUFS3 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P81601	NDUFS3 Antibody (YA1346)	WB	Human, Mouse
HY-P86511	NDUFS3 Antibody (YA6203)	WB, IHC-P, ICC/IF, IP, ELISA	Human, Mouse, Rat

Related Diseases

Diseases	Alias
Mitochondrial Complex I Deficiency, Nuclear Type 8	MC1DN8 Mitochondrial Complex 1 Deficiency, Nuclear Type 8 Nuclear Type Mitochondrial Complex I Deficiency 8
Mitochondrial Complex I Deficiency, Nuclear Type 1	Mitochondrial Complex I Deficiency Nadh:Q(1) Oxidoreductase Deficiency MC1DN1 Nadh-Coenzyme Q Reductase Deficiency Isolated Mitochondrial Respiratory Chain Complex I Deficiency Isolated Nadh-Coenzyme Q Reductase Deficiency Isolated Nadh-Coq Reductase Deficiency Isolated Nadh-Ubiquinone Reductase Deficiency Mitochondrial Nadh Dehydrogenase Component Of Complex I, Deficiency Of Nuclear Type Mitochondrial Complex I Deficiency 1 Isolated Complex I Deficiency Complex 1 Mitochondrial Respiratory Chain Deficiency Nadh Coenzyme Q Reductase Deficiency Complex I Mitochondrial Respiratory Chain Deficiency Deficiency Of Mitochondrial Nadh Dehydrogenase Component Of Complex I Nadh:Ubiquinone Oxidoreductase Deficiency Complex I, Mitochondrial Respiratory Chain, Deficiency Of
Leigh Syndrome With Leukodystrophy	Infantile Subacute Necrotizing Encephalopathy With Leukodystrophy Leigh Disease With Leukodystrophy
Leigh Syndrome	Leigh Disease Infantile Subacute Necrotizing Encephalopathy Leigh Syndrome Due To Mitochondrial Complex Iv Deficiency LS Sne Leigh'S Disease Leigh Syndrome Due To Mitochondrial Complex I Deficiency Necrotizing Encephalopathy, Infantile Subacute, Of Leigh Subacute Necrotizing Encephalomyelopathy Necrotizing Encephalopathy Infantile Subacute Of Leigh Leigh Syndrome Due To Mitochondrial Complex Iii Deficiency Infantile Necrotizing Encephalomyelopathy Juvenile Subacute Necrotizing Encephalomyelopathy Leigh'S Necrotizing Encephalopathy Subacute Necrotizing Encephalopathy Juvenile Subacute Necrotizing Encephalopathy Leigh Syndrome Due To Mitochondrial Complex Ii Deficiency Leigh Syndrome Due To Mitochondrial Complex V Deficiency Encephalopathy, Subacute Necrotizing, Infantile Encephalopathy, Subacute Necrotizing, Juvenile Maternally Inherited Leigh Syndrome Subacute Necrotising Encephalomyelopathy Subacute Necrotising Encephalopathy
Parathyroid Oncocytic Adenoma	Parathyroid Gland Oncocytic Adenoma
Cardiomyopathy, Familial Hypertrophic, 16	Hypertrophic Cardiomyopathy 16 CMH16 Cardiomyopathy, Hypertrophic, 16 Cardiomyopathy Familial Hypertrophic 16 Cardiomyopathy, Familial Hypertrophic 16 Cardiomyopathy, Hypertrophic, Familial, Type 16
Cardiomyopathy, Familial Hypertrophic, 18	Hypertrophic Cardiomyopathy 18 CMH18 Cardiomyopathy, Hypertrophic, 18 Cardiomyopathy Familial Hypertrophic 18 Cardiomyopathy, Familial Hypertrophic 18 Cardiomyopathy, Hypertrophic, Familial, Type 18
Cardiomyopathy, Familial Hypertrophic, 17	Hypertrophic Cardiomyopathy 17 CMH17 Cardiomyopathy, Hypertrophic, 17 Cardiomyopathy Familial Hypertrophic 17 Cardiomyopathy, Familial Hypertrophic 17 Cardiomyopathy, Hypertrophic, Familial, Type 17
Leukodystrophy	Leukodystrophies
Leber Hereditary Optic Neuropathy, Modifier Of	Leber Optic Atrophy Leber Hereditary Optic Neuropathy LHON Leber'S Hereditary Optic Neuropathy Leber Optic Atrophy, Susceptibility To Leber'S Optic Atrophy LOAM Loas Leber'S Disease Leber'S Optic Neuropathy Optic Atrophy, Hereditary, Leber Lhon, Modifier Of Optic Atrophy, Leber Type Hereditary Optic Neuroretinopathy Leber Hereditary Optic Atrophy Loa Optic Atrophy Leber Type Leber Hereditary Optic Neuropathy, Modifier Leber Hereditary Optic Neuropathy Susceptibility Modifier Of Leber Hereditary Optic Neuropathy Lebers Hereditary Optic Neuropathy Leber Congenital Amaurosis
Myopathy	Muscular Diseases Myopathies

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS09159	NDUFS3 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	NDUFS3	VGNC	VGNC:101366
Felis catus	NDUFS3	VGNC	VGNC:104315
Mus musculus	NDUFS3	MGD	MGI:1915599
Rattus norvegicus	NDUFS3	RGD	RGD:1309406
Bos taurus	NDUFS3	VGNC	VGNC:50228
Canis familiaris	NDUFS3	VGNC	VGNC:43708
Others	NDUFS3	NCBI