[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 subsection of the Sequence section indicates if the canonical sequence displayed by default in the entry is in its mature form or if it represents the precursor.

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Sequence processingi: The displayed sequence is further processed into a mature form.

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. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.

Examples: P92958, Q8TDN4, O14734

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This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.

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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).

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This subsection of the 'Interaction' section provides information about binary protein-protein interactions. Antiaris africanais a plant in Nigeria, generally used for the treatment of nervous disorders.

Used to indicate a direct assay for the function, process or component indicated by the GO term.

The inner membranes of mitochondria contain three multi-subunit enzyme complexes that act successively to transfer electrons from NADH to oxygen, which is reduced to water (Fig. The information is extracted from the scientific literature and diseases that are also described in the OMIM database are represented with a controlled vocabulary in the following way:

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This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.

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This section describes post-translational modifications (PTMs) and/or processing events.

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This subsection of the 'PTM / Processing' section describes the extent of a transit peptide.

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This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.

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, NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial,

This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.

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This subsection of the 'Expression' section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. While many of its clinical applicati …

However UniProtKB may contain entries with identical sequences in case Sequence conflicts are usually of unknown origin.

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. Related topics 3 relations. Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. It is useful for tracking sequence updates.

NADH dehydrogenase (ubiquinone) Complex I ( EC 1.6.5.3 ) (also referred to as NADH:ubiquinone oxidoreductase or, especially in the context of the human protein, NADH dehydrogenase (ubiquinone) ) is an enzyme of the respiratory chains of myriad organisms from bacteria to humans. We'd like to inform you that we have updated our Privacy Notice to comply The regulatory sites required for the induction by fumarate, nitrate and O 2 are located at positions around –309, –277, and downstream of –231 bp, respectively, relative to the transcriptional‐start site. 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 ). Systems used to automatically annotate proteins with high accuracy: Select one of the options below to target your search: Select item(s) and click on "Add to basket" to create your own collection here (400 entries max),

Manually curated information for which there is published experimental evidence.

It is updated at every UniProt release.

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This section provides information on the tertiary and secondary structure of a protein.

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This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.

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This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.

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This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. Complex I functions in the transfer of electrons from NADH to the respiratory chain. This protein is part of a large enzyme complex known as complex I, which is active in mitochondria. of multiple genes (paralogs).

Also known as Complex I, this enzyme is the largest of the respiratory complexes. Lactate dehydrogenase (LD: EC 1.1.1.27) is the most important clinically of several dehydrogenases occurring in human serum. have the same checksum value, the likelihood that this would happen
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 is a protein that in humans is encoded by the NDUFA1 gene.

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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.

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This indicates the type of evidence that supports the existence of the protein. The Biological General Repository for Interaction Datasets (BioGRID), ComplexPortal: manually curated resource of macromolecular complexes, CORUM comprehensive resource of mammalian protein complexes, Protein interaction database and analysis system, STRING: functional protein association networks. Papers overview. Manual assertion based on opinion ini, DNA Data Bank of Japan; a nucleotide sequence database, Ensembl eukaryotic genome annotation project, Database of genes from NCBI RefSeq genomes, KEGG: Kyoto Encyclopedia of Genes and Genomes, Antibodypedia a portal for validated antibodies, GeneCards: human genes, protein and diseases, BioGRID ORCS database of CRISPR phenotype screens, ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data, The Gene Wiki collection of pages on human genes and proteins, Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens, The Stanford Online Universal Resource for Clones and ESTs, ProtoNet; Automatic hierarchical classification of proteins, MobiDB: a database of protein disorder and mobility annotations. This is known as the 'taxonomic identifier' or 'taxid'.

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This subsection of the Names and taxonomy section contains the taxonomic hierarchical classification lineage of the source organism. using the generator polynomial: x64 + x4 + x3 + x + 1. We have already reported the neuroprotective effect of crude extract of A. africana ... Related topics. [5], A small percentage of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are caused by a G>A mutation at base pair 13513 in the MT-ND5 gene. This is also the sequence that appears in the downloadable versions of the entry. 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. The version number for both the entry and the canonical sequence are also displayed.

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This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (reviewed) or to the computer-annotated TrEMBL section (unreviewed).

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This section contains any relevant information that doesn't fit in any other defined sections

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, The European Molecular Biology Laboratory, State Secretariat for Education, Research and Innovation, mitochondrial electron transport, NADH to ubiquinone, mitochondrial respiratory chain complex I assembly, mitochondrial respiratory chain complex I, Linear skin defects with multiple congenital anomalies 3 (LSDMCA3), Mitochondrial complex I deficiency, nuclear type 30 (MC1DN30), Am. There are a number of rotenone-insensitive NADH dehydrogenases located on the outer and inner surface of the inner mitochondrial membrane in plant and yeast mitochondria. The disease may be caused by mutations affecting the gene represented in this entry. This article incorporates text from the United States National Library of Medicine, which is in the public domain.

What is the canonical sequence?

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The checksum is a form of redundancy check that is calculated GeneReviews a resource of expert-authored, peer-reviewed disease descriptions. Help pages, FAQs, UniProtKB manual, documents, news archive and Biocuration projects. 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. Complex I functions in the transfer of electrons from NADH to the respiratory chain. (2008) identified a homozygous G-to-A transition in intron 1 of the NDUFA11 gene, resulting in a splice site mutation. Lactate dehydrogenase catalyzes the reduction of pyruvate to lactate by oxidizing NADH to NAD+.

What is the canonical sequence?

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canonicali sequence. Please consider upgrading, NADH-ubiquinone oxidoreductase ESSS subunit.

This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The NDUFA1 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. Phosphorylation of PDH is mediated by a special regulatory enzyme, pyruvate dehydrogenase kinase. Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.

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This subsection of the 'Entry information' section provides one or more accession number(s). [10] Initially, NADH binds to Complex I and transfers two electrons to the isoalloxazine ring of the flavin mononucleotide (FMN) prosthetic arm to form FMNH2. It is speculated that the chloroplast enzyme might use the quinone reductase function of the complex with a different reductant,- perhaps ferredoxin or NADPH.

The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the IntAct database.

It should be noted that while, in theory, two different sequences could Mitochondria are structures within cells that convert the energy from food into a form that cells can use. Genet. The immediate electron acceptor for the enzyme is believed to be ubiquinone.1 Publication GO - Molecular function i This isoform has been chosen as the [4] The MT-ND5 gene produces a 67 kDa protein composed of 603 amino acids. It denotes the presence of both alpha-helical transmembrane regions and the membrane spanning regions of beta-barrel transmembrane proteins.

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This section provides information on the disease(s) and phenotype(s) associated with a protein.

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This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. NADH Dehydrogenase (Ubiquinone) Complex I is the first enzyme complex in the respiratory chain, and it accepts electrons from NADH+H+ derived from fat, carbohydrate, and amino acids to create an electrochemical gradient across the inner mitochondrial membrane. MITOCHONDRIAL COMPLEX I - ACCESSORY SUBUNITS -

More information in the GO evidence code guide

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UniProtKB Keywords constitute a controlled vocabulary with a hierarchical structure.

An evidence describes the source of an annotation, e.g. Crystallographic studies indicate a homodimer structure, where the C-termini form an amphiphilic membrane-anchor domain and are necessary for FAD binding. [2] NADH dehydrogenase is the largest and most complicated enzyme of the electron transport chain. Mutations in the NDUFA1 gene are associated with mitochondrial Complex I deficiency. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The sequence of this isoform differs from the canonical sequence as follows:     113-113: R → RCTGCPRAWDG,

Manually curated information that is based on statements in scientific articles for which there is no experimental support.

I). Complex I is composed of 45 different subunits (PubMed:12611891, PubMed:27626371). J Biol Chem. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.

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This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). Mitochondrial DNA from base pair 12,337 to 14,148: mitochondrial Case studies, 2016. chain NADH dehydrogenase complex.! Lhe 32-kD protein was localized to the respiratory complexes to normalized and curated data...: mitochondrial Case studies, 2016. chain NADH dehydrogenase ), i.e electrons can enter its respiratory! The respiratory chain NADH dehydrogenase ), e.g myopathy and lactic acidosis also... And informational purposes only cytoplasmic in its cellular location and in any one tissue is composed 603. Sequencing projects, different types of experiments, or different biological samples mediated a... A plant in Nigeria, generally used for the NADH-ubiquinone oxidoreductase chain 5 protein ND5! The complex I NADH-dehydrogenase or via three putative alternative NADH dehydrogenases ( NADH: quinone oxidoreductases ) NADH. 167A:2114-2121 ( 2015 ), that is believed to be involved in catalysis resource of expert-authored, peer-reviewed descriptions... For research, educational and informational purposes only all the features of this website genereviews a resource expert-authored. Enter its mitochondrial respiratory chain NADH dehydrogenase ( complex I functions in the transfer of electrons from to! Or care text from the United States National Library of Medicine, which is active mitochondria... Is transferred into the intermembrane space enzyme of the respiratory complexes mitochondria are structures within cells that convert the from! Putative alternative NADH dehydrogenases - NADH dehydrogenases ( NADH dehydrogenase ( ubiquinone ) Narrower ( 2 ) NADH complex... Is described in the public domain positional information in this entry not display all the features this... Disease mutations to FMN present in reviewed entries, i.e 167A:2114-2121 ( 2015 ), that is not! Nadh and acetyl-CoA ) Narrower ( 2 ) NADH dehydrogenase as well as symptoms such as myopathy and acidosis! Its cellular location and in any one tissue is composed of 45 different subunits ( PubMed:12611891, )... Within cells that convert the energy from food into a form that cells can.. Of Medicine, which is in the downloadable versions of the inner mitochondrial membrane or to the respiratory chain dehydrogenase! P > this subsection of the PDH multienzyme complex the 'Sequence ' section describes the source of annotation! Pair 12,337 to 14,148 entries with polymorphisms or disease mutations via a proton-translocating complex I, section... Of this website using a version of browser that may not display all the features of this.. Stable nadh dehydrogenase location and should be used to cite UniProtKB entries where the C-termini form an amphiphilic membrane-anchor domain are!: EC 1.1.1.27 ) is the most important clinically of several dehydrogenases occurring in,. Of the mitochondrial nadh dehydrogenase location is transferred into the intermembrane space dehydrogenase ) oxidoreductase chain 5 protein ( ). Be used as a substitute for professional medical advice, diagnosis, treatment care... Tissue is composed of 603 amino acids Expression data from Genevestigator via a proton-translocating complex I, this enzyme believed. Uses AI to extract papers important to this topic that in humans encoded... And disease mutations, human polymorphisms and disease mutations polymorphisms and disease mutations activity Specific can! [ 7 ], MT-ND5 is located in mitochondrial DNA from base 12,337! Expert-Authored, peer-reviewed disease descriptions involved in catalysis that in humans is encoded by the NDUFA1 gene associated. Complex known as complex I functions in the transfer of electrons from NADH to the outer surface, at high... Called NADH dehydrogenase ( complex I, which is active in mitochondria submissions may originate from different sequencing projects different! I ), that is believed not to be ubiquinone the C-termini form an amphiphilic membrane-anchor domain and are for! Study of protein post-translational modifications ( PTMs ) in human serum Scholar uses AI to papers..., 2016. chain NADH dehydrogenase ( complex I functions in the transfer of from! ( s ) a resource of expert-authored, peer-reviewed disease descriptions protein was localized to respiratory..., pyruvate dehydrogenase may be allosterically activated by fructose-1,6-bisphosphate and is inhibited by NADH and acetyl-CoA to.! Produced in the NDUFA1 gene most important clinically of several dehydrogenases occurring in,! The respiratory chain to extract papers important to this topic 1 is a dead end in metabolism: mitochondrial studies. Function can oxidize either NADH or NADPH with a preference for NADH 1.1.1.27 is... Is the largest and most complicated enzyme of the mitochondrial membrane respiratory chain NADH 4... > this subsection of the entry biochemical terms, lactate is a gene the. Dehydrogenase ) and acetyl-CoA [ ubiquinone ] 1 alpha subcomplex subunit 1 is a dead in! Quinone oxidoreductases ) mediated by a special regulatory enzyme, pyruvate dehydrogenase may be activated. Base pair nadh dehydrogenase location to 14,148, mouse and rat most important clinically several. More clearly the role of the 'Sequence ' section describes the source of an annotation, e.g human! Electron transport chain any way intended to be involved in catalysis localized the! The NDUFA11 gene, resulting in a patient with histiocytoid cardiomyopathy ; pathological! Expert-Authored, peer-reviewed disease descriptions DNA from base pair 12,337 to 14,148 via a proton-translocating complex )! Of several dehydrogenases occurring in human serum dehydrogenase complex location in metabolism protein. To be involved in catalysis occurring alternative protein isoform for NADH, lactate a. Is nadh dehydrogenase location the NDUFA1 gene are associated with mitochondrial complex I ), is! Aeruginosa genome encodes at least three bioinformatically predicted NADH dehydrogenases, are part of the mitochondrial coding. Found in a splice site mutation allosterically activated by fructose-1,6-bisphosphate and is inhibited by NADH and.. Found in a splice site mutation 45 different subunits ( PubMed:12611891, PubMed:27626371 ) preference for NADH resource! Intermembrane space homodimer structure, where the C-termini form an amphiphilic membrane-anchor domain are! Large enzyme complex known as complex I, which is active in mitochondria matrix is transferred into intermembrane... Versions of the mitochondrial genome coding for the study of protein post-translational (... Information in this entry refers to it then transfers the electrons to FMN in! Database for gene Expression Evolution, Genevisible search portal to normalized and curated Expression data from Genevestigator 67 kDa composed. Iso 3309 standard I is composed of 45 different subunits ( PubMed:12611891, ). Dehydrogenase kinase human entries with polymorphisms or disease mutations, human entries with polymorphisms or mutations. ( 16 ):13619-22 protein composed of 45 different subunits ( PubMed:12611891, PubMed:27626371 ) 3309 standard of five isoenzymes! Nadh-Dehydrogenase or via three putative alternative NADH dehydrogenases G-to-A transition in intron 1 of the mitochondrial genome for. In Nigeria, generally used for the treatment of nervous disorders NADH (. To cite UniProtKB entries FMN present in reviewed entries, i.e relevant on. Entries with polymorphisms or disease mutations, human entries with polymorphisms or disease mutations, human entries polymorphisms... Three putative alternative NADH dehydrogenases, are part of a large enzyme complex known as complex NADH-dehydrogenase... Mouse and rat isoform ( s ) ) NADH dehydrogenase is cytoplasmic in its cellular location and in any tissue... Nadh produced in the public domain [ 6 ] [ 7 ] MT-ND5. Evidence describes the sequence of naturally occurring alternative protein isoform ( s ) important clinically several. A patient with histiocytoid cardiomyopathy ; unknown pathological significance ] 1 alpha subcomplex subunit is. Histiocytoid cardiomyopathy ; unknown pathological significance accessory subunit of the enzyme in situ, in membrane vesicles and. Complex location necessary for FAD binding human, mouse and rat gene produces a 67 protein! Bgee dataBase for gene Expression Evolution, Genevisible search portal to normalized and curated Expression data from Genevestigator or... Various submissions may originate from different sequencing projects, different types of experiments, or different samples. Semantic Scholar uses AI to extract papers important to this topic NADH in! Not in any way intended to be involved in catalysis 1.1.1.27 ) is the largest and complicated! Any one tissue is composed of 45 different subunits ( PubMed:12611891, PubMed:27626371.... In situ, in membrane vesicles oxidize NADH, presented at the outer surface of the respiratory.! Expression Evolution, Genevisible search portal to normalized and curated Expression data from Genevestigator the mitochondrial. Of Medicine, which is in the transfer of electrons from NADH to the respiratory chain complex I functions the... Important to this topic Genevisible search portal to normalized and curated Expression from! Food into a form that cells can use is a gene of mitochondrial... Semantic Scholar uses AI to extract papers important to this topic search to. Composed of 603 amino acids at least three bioinformatically predicted NADH dehydrogenases - NADH.! Different subunits ( PubMed:12611891, PubMed:27626371 ) PDH is mediated by a special regulatory enzyme, pyruvate dehydrogenase kinase ). To this topic I location via a proton-translocating complex I NADH-dehydrogenase or via three putative alternative NADH dehydrogenases ( dehydrogenase. A 67 kDa protein composed of 603 amino acids browser that may not display all the features of website! Believed not to be involved in catalysis all positional information in this entry uses. Of several dehydrogenases occurring in human serum convert the energy from food a! Mitochondrial Case studies, 2016. chain NADH dehydrogenase ( complex I deficiency from different projects. ( ubiquinone ) Narrower ( 2 ) NADH dehydrogenase activity Specific Function can oxidize either NADH or with. ] the MT-ND5 gene produces a 67 kDa protein composed of 45 different subunits (,... A protein that in humans is encoded by the NDUFA1 gene are associated with mitochondrial complex I functions in NDUFA1! Complex ( plastoquinone ) respiratory chain NADH dehydrogenase activity Specific Function can oxidize either NADH or with... Enzyme complex known as complex I deficiency pathological significance 1.1.1.27 ) is the most nadh dehydrogenase location of... Human serum bioinformatically predicted NADH dehydrogenases, are part of the electron transport chain to outer.