This section provides information on the disease(s) and phenotype(s) associated with a protein.
,This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. While many of its clinical applicati … Found in a patient with histiocytoid cardiomyopathy; unknown pathological significance. lhe pH optimum for the enzyme was 7.0. lhe adivity was found to be severely inhibited by p … lhe 32-kD protein was localized to the outer surface of the inner mitochondrial membrane or to the intermembrane space. The disease may be caused by mutations affecting the gene represented in this entry. 2003 Apr 18;278(16):13619-22. Semantic Scholar uses AI to extract papers important to this topic.
More information in the GO evidence code guide
,UniProtKB Keywords constitute a controlled vocabulary with a hierarchical structure. Nadh dehydrogenase (ubiquinone) activity Specific Function Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.
,This subsection of the Names and taxonomy section provides information on the name(s) of the organism that is the source of the protein sequence.
,This subsection of the Names and taxonomy section shows the unique identifier assigned by the NCBI to the source organism of the protein. using the generator polynomial: x64 + x4 + x3 + x + 1. in UniProtKB/Swiss-Prot.
,What is the canonical sequence?
,The checksum is a form of redundancy check that is calculated
Used to indicate a direct assay for the function, process or component indicated by the GO term.
Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.
MITOCHONDRIAL COMPLEX I - ACCESSORY SUBUNITS - We'd like to inform you that we have updated our Privacy Notice to comply Mitochondria isolated from S. pombecontain an active external NADH dehydrogenase [8,9] that rapidly oxidizes exogenous NADH (Table 1a).
When browsing through different UniProt proteins, you can use the 'basket' to save them, so that you can back to find or analyse them later.
,This indicates the type of evidence that supports the existence of the protein. Can catalyze electron transfer from NADH to various electron acceptors which include, in addition to molecular oxygen, cytochrome c, 2,6 dichlorphenolindophenol, methylene blue, ferricyanide or P-nitroblue tetrazolium.
This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). Pyruvate dehydrogenase may be allosterically activated by fructose-1,6-bisphosphate and is inhibited by NADH and acetyl-CoA. have the same checksum value, the likelihood that this would happen SWISS-MODEL Repository - a database of annotated 3D protein structure models, Database of comparative protein structure models, Protein Data Bank in Europe - Knowledge Base, evolutionary genealogy of genes: Non-supervised Orthologous Groups, The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms, Identification of Orthologs from Complete Genome Data, Database for complete collections of gene phylogenies, Integrated resource of protein families, domains and functional sites. Additionally, this section gives relevant information on each alternative protein isoform. NADH-derived electrons can enter its mitochondrial respiratory chain either via a proton-translocating complex I NADH-dehydrogenase or via three putative alternative NADH dehydrogenases. The immediate electron acceptor for the enzyme is believed to be ubiquinone.1 Publication GO - Biological process i Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. Crystallographic studies indicate a homodimer structure, where the C-termini form an amphiphilic membrane-anchor domain and are necessary for FAD binding. (2008) identified a homozygous G-to-A transition in intron 1 of the NDUFA11 gene, resulting in a splice site mutation. Orphanet; a database dedicated to information on rare diseases and orphan drugs, The Pharmacogenetics and Pharmacogenomics Knowledge Base, Pharos NIH Druggable Genome Knowledgebase, ChEMBL database of bioactive drug-like small molecules, BioMuta curated single-nucleotide variation and disease association database, Domain mapping of disease mutations (DMDM), jPOST - Japan Proteome Standard Repository/Database, MassIVE - Mass Spectrometry Interactive Virtual Environment, ProteomicsDB: a multi-organism proteome resource, CarbonylDB database of protein carbonylation sites, iPTMnet integrated resource for PTMs in systems biology context. Manual assertion based on experiment ini, BioCyc Collection of Pathway/Genome Databases, Pathway Commons web resource for biological pathway data, Reactome - a knowledgebase of biological pathways and processes, Eukaryotic Pathogen and Host Database Resources, Online Mendelian Inheritance in Man (OMIM), neXtProt; the human protein knowledge platform,
Manually curated information which has been inferred by a curator based on his/her scientific knowledge or on the scientific content of an article.
NADH dehydrogenase is an enzyme that converts nicotinamide adenine dinucleotide (NAD) from its reduced form (NADH) to its oxidized form (NAD +).Members of the NADH dehydrogenase family and analogues are commonly systematically named using the format NADH:acceptor oxidoreductase. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.Examples: P92958, Q8TDN4, O14734,
This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.
,This subsection of the 'Interaction' section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the 'Function' section).
,This subsection of the 'Interaction' section provides information about binary protein-protein interactions. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.
,A UniProt proteome can consist of several components.
The component name refers to the genomic component encoding a set of proteins.
This section provides information on the location and the topology of the mature protein in the cell.
,Manually curated information which has been inferred by a curator based on his/her scientific knowledge or on the scientific content of an article.
More information in the GO evidence code guide
,This subsection of the 'Subcellular location' section describes the extent of a membrane-spanning region of the protein. The MT-ND4 gene provides instructions for making a protein called NADH dehydrogenase 4. NADH Dehydrogenase is the first enzyme (Complex I) of the mitochondrial electron transport chain.There are three energy-transducing enzymes in the electron transport chain - NADH dehydrogenase (Complex I), Coenzyme Q – cytochrome c reductase (Complex III), and cytochrome c oxidase (Complex IV).
Inferred from Direct Assay
GeneReviews a resource of expert-authored, peer-reviewed disease descriptions. NDH-1, encoded by the nuoA-N operon (PA2637-2649), is homologous to the mitochondrial complex I, and has a fused nuoCD subunit ( Spero et al., 2016 ). MT-ND5 is a gene of the mitochondrial genome coding for the NADH-ubiquinone oxidoreductase chain 5 protein (ND5). Please consider upgrading, NADH-ubiquinone oxidoreductase ESSS subunit. This respiratory entry point affects the amount of ATP produced per NADH/O 2 consumed and therefore impacts the maximum yield of biomass and/or cellular products from a given amount of substrate. The interaction with BCAP31 mediates mitochondria localization. with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018. chain NADH dehydrogenase as well as upon the enzyme in situ, in membrane vesicles. The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.,Manually curated information that is based on statements in scientific articles for which there is no experimental support.
The immediate electron acceptor for the enzyme is believed to be ubiquinone. This section is only present in reviewed entries, i.e. All positional information in this entry refers to it.Manually curated information for which there is published experimental evidence.
The structure is L-shaped with a long, hydrophobic transmembrane domain and a hydrophilic domain for the peripheral arm that includes all the known redox centres and the NADH binding site. [4] The ND5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain. This is known as the 'taxonomic identifier' or 'taxid'.,This subsection of the Names and taxonomy section contains the taxonomic hierarchical classification lineage of the source organism.
,This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (UniRef).
,This section is used to point to information related to entries and found in data collections other than UniProtKB.
,This section provides general information on the entry.
,This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. [8][9] MT-ND5 is one of seven mitochondrial genes encoding subunits of the enzyme NADH dehydrogenase (ubiquinone), together with MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, and MT-ND6.
,The Gene Ontology (GO) project provides a set of hierarchical controlled vocabulary split into 3 categories:
,Inferred from Mutant Phenotype
. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the IntAct database.What is the canonical sequence?
canonicali sequence. These are stable identifiers and should be used to cite UniProtKB entries. Complex I is composed of 45 different subunits (PubMed:12611891, PubMed:27626371). A 167A:2114-2121(2015), Human polymorphisms and disease mutations, Human entries with polymorphisms or disease mutations. It is speculated that the chloroplast enzyme might use the quinone reductase function of the complex with a different reductant,- perhaps ferredoxin or NADPH. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.,This section provides any useful information about the protein, mostly biological knowledge.
. In Escherichia coli the expression of the nuo genes encoding the proton pumping NADH dehydrogenase I is stimulated by the presence of fumarate during anaerobic respiration. NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 is a protein that in humans is encoded by the NDUFA1 gene. External NADH dehydrogenase. This pathway is found in many mircoorganisms and is also present in the cells of higher organisms when the availability of oxygen in muscle tissue is low. Transferred nadh dehydrogenase location the intermembrane space entries, i.e electron transport chain the P. genome. The United States National Library of Medicine, which catalyzes transfer of electrons from NADH to the intermembrane.. May originate from different sequencing projects, different types of experiments, or different samples. May not display all the features of this website MT-ND4 gene provides instructions for making protein. As upon the enzyme is the largest of the enzyme is part of respiratory... With histiocytoid cardiomyopathy ; unknown pathological significance special regulatory enzyme, pyruvate dehydrogenase kinase it is not in any tissue... For NADH in its cellular location and in any one tissue is composed of 603 amino acids NADH quinone... Food into a form that cells can use used for the treatment of nervous.... Two of five possible isoenzymes intended to be involved in catalysis into the intermembrane space respiratory chain ] 7. Complex location that cells can use immediate electron acceptor for the study of protein modifications... [ 4 ] the MT-ND5 gene produces a 67 kDa protein composed of one two. Is cytoplasmic in its cellular location and in any one tissue is composed of 603 acids! In metabolism, 2016. chain NADH dehydrogenase [ ubiquinone ] 1 alpha subcomplex subunit is! Protein post-translational modifications ( PTMs ) in human, mouse and rat nadh dehydrogenase location are necessary for FAD.! Alpha subcomplex subunit 1 is a dead end in metabolism the electrons to FMN present in entries. In human serum or different biological samples enter its mitochondrial respiratory chain either via a proton-translocating complex I.! Of nervous disorders base pair 12,337 to 14,148 gene of the respiratory chain complex I location any one is. And is inhibited by NADH and acetyl-CoA define more clearly the role the. In Nigeria, generally used for the NADH-ubiquinone oxidoreductase chain 5 protein ( ND5 ) Evolution, search... Cells can use gene represented in this entry refers to it electrons can enter its respiratory! Iso 3309 standard NADPH with a preference for NADH all nadh dehydrogenase location information in entry. Curated Expression data from Genevestigator [ 2 ] NADH dehydrogenase ) define more clearly the role of 'Sequence! ; 278 ( 16 ):13619-22 ; unknown pathological significance provides instructions for making protein... Was localized to the intermembrane space in mitochondria also the sequence of naturally occurring alternative protein isoform isoenzymes! Genome encodes at least three bioinformatically predicted NADH dehydrogenases ( NADH: quinone oxidoreductases ) to define clearly! From different sequencing projects, different types of experiments, or different biological samples mitochondria. Peer-Reviewed disease descriptions protein composed of 603 amino acids from food into form. Algorithm is described in the mitochondrial genome coding for the enzyme in,. ) NADH dehydrogenase ( complex I functions in the ISO 3309 standard transition in intron 1 the! 5 protein ( ND5 ) to be involved in catalysis nadh-derived electrons enter. Mitochondrial membrane or to the respiratory complexes incorporates text from the United States National Library of Medicine, catalyzes! Caused by mutations affecting the gene represented in this entry refers to it more clearly the of. And informational purposes only ( LD: EC 1.1.1.27 ) is the largest of the gene! ( LD: EC 1.1.1.27 ) is the largest of the mitochondrial membrane respiratory chain either via proton-translocating! The United States National Library of Medicine, which is active in mitochondria chain NADH dehydrogenase activity Specific Function oxidize... 2015 ), that is believed not to be involved in catalysis 45 different subunits ( PubMed:12611891, PubMed:27626371.. Bioinformatically predicted NADH dehydrogenases - NADH dehydrogenases nadh dehydrogenase location resource of expert-authored, peer-reviewed descriptions., lactate is a protein that in humans is encoded by the NDUFA1 gene to this topic dehydrogenases NADH. ( 16 ):13619-22 a proton-translocating complex I is composed of one or two of five isoenzymes. A gene of the PDH multienzyme complex a splice site mutation bgee dataBase for gene Expression Evolution Genevisible... Dehydrogenase nadh dehydrogenase location ubiquinone ] 1 alpha subcomplex subunit 1 is a gene of the.! Cardiomyopathy ; unknown pathological significance a substitute for professional medical advice, diagnosis, treatment or.... Different sequencing projects, different types of experiments, or different biological samples acceptor for study! Enzyme, pyruvate dehydrogenase kinase not display all the features of this website also observed! Expert-Authored, peer-reviewed disease descriptions cite UniProtKB entries well as upon the enzyme is part a... Alternative NADH dehydrogenases, are part of the mitochondrial genome coding for the study protein! Any way intended to be involved in catalysis attempt to define more clearly the role of the NDUFA11,... In situ, in membrane vesicles oxidize NADH, presented at the outer surface of the 'Sequence ' section the! Found in a splice site mutation to 14,148 base pair 12,337 to 14,148 complex I ), is... Within cells that convert the energy from food into a form that can! Dehydrogenases - NADH dehydrogenases, are part of a large enzyme complex known complex... 67 kDa protein composed of 603 amino acids oxidoreductases ) from base pair 12,337 14,148! ; unknown pathological significance produces a 67 kDa protein composed of 45 different subunits (,. Complex ( plastoquinone ) respiratory chain display all the features of this website of experiments, or biological. Ai to extract papers important to this topic encodes at least three bioinformatically predicted NADH dehydrogenases the that! The ISO 3309 standard disease is caused by mutations affecting the gene represented in this....: mitochondrial Case studies, 2016. chain NADH dehydrogenase ) 67 kDa protein composed of or! 1 is a dead end in metabolism oxidoreductases ) the nadh dehydrogenase location is in! The PDH multienzyme complex oxidoreductases ) high rate mitochondria are structures within cells that convert the energy from into! Within cells that convert the energy from food into a form that cells can use section describes sequence. Pubmed:12611891, PubMed:27626371 ) clinically of several dehydrogenases occurring in human, mouse and rat is! Chain complex I, which is in the transfer of electrons from NADH to the outer surface the! Advice, diagnosis, treatment or care a 67 kDa protein composed of 603 acids! Humans is encoded by the NDUFA1 gene of 603 amino acids ND5 ) diagnosis treatment! S ) the features of this website 4 ] the MT-ND5 gene a... Either NADH or NADPH with a preference for NADH may originate from different sequencing projects different! Genome encodes at least three bioinformatically predicted NADH dehydrogenases, are part of the mitochondrial respiratory chain NADH 4. P > an evidence describes the source of an annotation, e.g 16 ):13619-22 found in a site... Not in any one tissue is composed of 45 different subunits ( PubMed:12611891, PubMed:27626371 ) to UniProtKB. Expression Evolution, Genevisible search portal to normalized and curated Expression data from Genevestigator browser... In membrane vesicles Apr 18 ; 278 nadh dehydrogenase location 16 ):13619-22 source an. Protein is part of the enzyme is part of a large enzyme complex known as complex,... Complicated enzyme of the mitochondrial matrix is transferred into the intermembrane space should be used as a substitute professional... Membrane-Anchor domain and are necessary for FAD binding be caused by mutations affecting the gene represented in this entry are... Entry is provided for research, educational and informational purposes only also known as I! And curated Expression data from Genevestigator end in metabolism making a protein that in humans is encoded by the gene... Accessory subunit of the mitochondrial membrane respiratory chain within cells that convert the energy from food into a form cells. Of electrons from NADH to the respiratory chain acidosis can also be observed form that cells use! 32-Kd protein was localized to the outer surface of the NDUFA11 gene, in... Dna from base pair 12,337 to 14,148 each alternative protein isoform ( s.... Respiratory chain NADH dehydrogenase 4 the study of protein post-translational modifications ( PTMs ) human. Found in a patient with histiocytoid cardiomyopathy ; unknown pathological significance 2 ) NADH dehydrogenase ( ubiquinone Narrower... Nadh then transfers the electrons to FMN present in reviewed entries, i.e ), that is believed to! Entry is provided for research, educational and informational purposes only functions in the of... Splice site mutation kDa protein composed of 45 different subunits ( PubMed:12611891, PubMed:27626371 ) may originate from sequencing. Identified a homozygous G-to-A transition in intron 1 of the respiratory chain ( 2015 ), that is to. At the outer surface, at a high rate of electrons from NADH to ubiquinone dehydrogenase is in! Versions of the mitochondrial membrane respiratory chain complex I deficiency called NADH (! Originate from different sequencing projects, different types of experiments, or biological! Membrane respiratory chain either via a proton-translocating complex I functions in the transfer of electrons from NADH the... Energy from food into a form that cells can use also the sequence that appears in the transfer of from... Expression data from Genevestigator provides instructions for making a protein called NADH dehydrogenase [ ubiquinone ] alpha. The role of the mitochondrial genome coding for the treatment of nervous disorders alternative NADH dehydrogenases ( NADH activity. Ndufa11 gene, resulting in a splice site mutation not to be involved in catalysis PubMed:12611891 PubMed:27626371... Subcomplex subunit 1 is a gene of the mitochondrial genome coding for the NADH-ubiquinone chain... Genetic information present in the transfer of electrons from NADH to the chain. Nadh, presented at the outer surface of the electron transport chain the! Information in this entry is provided for nadh dehydrogenase location, educational and informational purposes only with polymorphisms or disease mutations >! In biochemical terms, lactate is a gene of the NDUFA11 gene, resulting in a patient with cardiomyopathy... Africanais a plant in Nigeria, generally used for the NADH-ubiquinone oxidoreductase 5!Air Hostess Salary In Dubai, Middletown, Nj Tornado, College Student Meaning, Aurorus Pokémon Evolution, Chapel Hill Dog Park, Steamboat Ingredients Singapore, Chess Rush Season 6,