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Protein

Malate dehydrogenase, mitochondrial

Gene

MDH2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Enzyme activity is enhanced by acetylation.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei57NAD1 Publication1
Binding sitei104SubstratePROSITE-ProRule annotation1 Publication1
Binding sitei110SubstratePROSITE-ProRule annotation1 Publication1
Binding sitei117NAD1 Publication1
Binding sitei142SubstratePROSITE-ProRule annotation1 Publication1
Binding sitei176SubstratePROSITE-ProRule annotation1 Publication1
<p>This subsection of the ‘Function’ section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei200Proton acceptorBy similarity1
Binding sitei251NAD1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi31 – 37NAD1 Publication7
Nucleotide bindingi140 – 142NAD1 Publication3

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionOxidoreductase
Biological processTricarboxylic acid cycle
LigandNAD

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS07366-MONOMER

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-70263 Gluconeogenesis
R-HSA-71403 Citric acid cycle (TCA cycle)

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Malate dehydrogenase, mitochondrial (EC:1.1.1.371 Publication)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:MDH2
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 7

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...
EuPathDBi
HostDB:ENSG00000146701.11

Human Gene Nomenclature Database

More...
HGNCi
HGNC:6971 MDH2

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
154100 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P40926

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Epileptic encephalopathy, early infantile, 51 (EIEE51)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of epileptic encephalopathy, a heterogeneous group of severe childhood onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. EIEE51 is an autosomal recessive form characterized by onset of intractable seizures and hypotonia in the first days or weeks of life, and severely delayed psychomotor development.
See also OMIM:617339
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_07800137G → R in EIEE51; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs782308462EnsemblClinVar.1
Natural variantiVAR_078002133P → L in EIEE51; decreased protein abundance; strong decrease in malate dehydrogenase activity; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs375002796EnsemblClinVar.1
Natural variantiVAR_078003207P → L in EIEE51; decreased protein abundance; strong decrease in malate dehydrogenase activity; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs1057519566Ensembl.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi185K → R: No activation of enzyme activity on treatment with TSA or NAM; when associated with R-301; R-307 and R-314. 1 Publication1
Mutagenesisi301K → R: No activation of enzyme activity on treatment with TSA or NAM; when associated with R-185; R-307 and R-314. 1 Publication1
Mutagenesisi307K → R: No activation of enzyme activity on treatment with TSA or NAM; when associated with R-185; R-301 and R-314. 1 Publication1
Mutagenesisi314K → R: No activation of enzyme activity on treatment with TSA or NAM; when associated with R-185; R-301 and R-307. 1 Publication1

Keywords - Diseasei

Disease mutation, Epilepsy

Organism-specific databases

DisGeNET

More...
DisGeNETi
4191

MalaCards human disease database

More...
MalaCardsi
MDH2
MIMi617339 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000146701

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
29072 Hereditary pheochromocytoma-paraganglioma

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA30716

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...
ChEMBLi
CHEMBL5917

Drug and drug target database

More...
DrugBanki
DB04272 Citric Acid
DB00157 NADH

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
MDH2

Domain mapping of disease mutations (DMDM)

More...
DMDMi
215274114

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 24MitochondrionBy similarityAdd BLAST24
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000001862825 – 338Malate dehydrogenase, mitochondrialAdd BLAST314

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section specifies the position and type of each covalently attached glycan group (mono-, di-, or polysaccharide).<p><a href='/help/carbohyd' target='_top'>More...</a></p>Glycosylationi33O-linked (GlcNAc) serineBy similarity1
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei78N6-acetyllysine; alternateBy similarity1
Modified residuei78N6-succinyllysine; alternateBy similarity1
Modified residuei91N6-acetyllysine; alternateBy similarity1
Modified residuei91N6-succinyllysine; alternateBy similarity1
Modified residuei165N6-acetyllysineCombined sources1
Modified residuei185N6-acetyllysine; alternateCombined sources1 Publication1
Modified residuei185N6-succinyllysine; alternateBy similarity1
Modified residuei203N6-succinyllysineBy similarity1
Modified residuei215N6-acetyllysine; alternateBy similarity1
Modified residuei215N6-succinyllysine; alternateBy similarity1
Modified residuei239N6-acetyllysine; alternateBy similarity1
Modified residuei239N6-malonyllysine; alternateBy similarity1
Modified residuei239N6-succinyllysine; alternateBy similarity1
Modified residuei246PhosphoserineCombined sources1
Modified residuei269N6-succinyllysineBy similarity1
Modified residuei296N6-acetyllysine; alternateBy similarity1
Modified residuei296N6-succinyllysine; alternateBy similarity1
Modified residuei301N6-acetyllysine; alternateCombined sources1 Publication1
Modified residuei301N6-succinyllysine; alternateBy similarity1
Modified residuei307N6-acetyllysine; alternate1 Publication1
Modified residuei307N6-malonyllysine; alternate1 Publication1
Modified residuei307N6-succinyllysine; alternateBy similarity1
Modified residuei314N6-acetyllysine; alternateCombined sources1 Publication1
Modified residuei314N6-succinyllysine; alternateBy similarity1
Modified residuei324N6-acetyllysine; alternateBy similarity1
Modified residuei324N6-succinyllysine; alternateBy similarity1
Modified residuei326PhosphoserineCombined sources1
Modified residuei328N6-acetyllysine; alternateBy similarity1
Modified residuei328N6-succinyllysine; alternateBy similarity1
Modified residuei329N6-acetyllysine; alternateCombined sources1
Modified residuei329N6-malonyllysine; alternateBy similarity1
Modified residuei335N6-acetyllysine; alternateCombined sources1
Modified residuei335N6-succinyllysine; alternateBy similarity1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Acetylation is enhanced by up to 67% after treatment either with trichostin A (TSA) or with nicotinamide (NAM) with the appearance of tri- and tetraacetylations. Glucose also increases acetylation by about 60%.1 Publication

Keywords - PTMi

Acetylation, Glycoprotein, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...
EPDi
P40926

MaxQB - The MaxQuant DataBase

More...
MaxQBi
P40926

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
P40926

PeptideAtlas

More...
PeptideAtlasi
P40926

PRoteomics IDEntifications database

More...
PRIDEi
P40926

ProteomicsDB human proteome resource

More...
ProteomicsDBi
55386

Consortium for Top Down Proteomics

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TopDownProteomicsi
P40926-1 [P40926-1]

2D gel databases

DOSAC-COBS 2D-PAGE database

More...
DOSAC-COBS-2DPAGEi
P40926

REPRODUCTION-2DPAGE

More...
REPRODUCTION-2DPAGEi
IPI00291006
P40926

University College Dublin 2-DE Proteome Database

More...
UCD-2DPAGEi
P40926

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
P40926

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
P40926

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
P40926

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000146701 Expressed in 244 organ(s), highest expression level in vastus lateralis

CleanEx database of gene expression profiles

More...
CleanExi
HS_MDH2

ExpressionAtlas, Differential and Baseline Expression

More...
ExpressionAtlasi
P40926 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...
Genevisiblei
P40926 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA019714
HPA019716
HPA019848
HPA026720

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> 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 <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer.1 Publication

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
110356, 64 interactors

Protein interaction database and analysis system

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IntActi
P40926, 25 interactors

Molecular INTeraction database

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MINTi
P40926

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000327070

Chemistry databases

BindingDB database of measured binding affinities

More...
BindingDBi
P40926

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1338
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P40926

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P40926

Database of comparative protein structure models

More...
ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...
EvolutionaryTracei
P40926

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the LDH/MDH superfamily. MDH type 1 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG1494 Eukaryota
COG0039 LUCA

Ensembl GeneTree

More...
GeneTreei
ENSGT00390000016686

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...
HOGENOMi
HOG000213792

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG001662

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P40926

KEGG Orthology (KO)

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KOi
K00026

Identification of Orthologs from Complete Genome Data

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OMAi
QCTPKVE

Database of Orthologous Groups

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OrthoDBi
EOG091G0AVO

Database for complete collections of gene phylogenies

More...
PhylomeDBi
P40926

TreeFam database of animal gene trees

More...
TreeFami
TF300834

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
3.90.110.10, 1 hit

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR001557 L-lactate/malate_DH
IPR022383 Lactate/malate_DH_C
IPR001236 Lactate/malate_DH_N
IPR015955 Lactate_DH/Glyco_Ohase_4_C
IPR001252 Malate_DH_AS
IPR010097 Malate_DH_type1
IPR036291 NAD(P)-bd_dom_sf

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF02866 Ldh_1_C, 1 hit
PF00056 Ldh_1_N, 1 hit

PIRSF; a whole-protein classification database

More...
PIRSFi
PIRSF000102 Lac_mal_DH, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF51735 SSF51735, 1 hit
SSF56327 SSF56327, 1 hit

TIGRFAMs; a protein family database

More...
TIGRFAMsi
TIGR01772 MDH_euk_gproteo, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00068 MDH, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P40926-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MLSALARPAS AALRRSFSTS AQNNAKVAVL GASGGIGQPL SLLLKNSPLV
60 70 80 90 100
SRLTLYDIAH TPGVAADLSH IETKAAVKGY LGPEQLPDCL KGCDVVVIPA
110 120 130 140 150
GVPRKPGMTR DDLFNTNATI VATLTAACAQ HCPEAMICVI ANPVNSTIPI
160 170 180 190 200
TAEVFKKHGV YNPNKIFGVT TLDIVRANTF VAELKGLDPA RVNVPVIGGH
210 220 230 240 250
AGKTIIPLIS QCTPKVDFPQ DQLTALTGRI QEAGTEVVKA KAGAGSATLS
260 270 280 290 300
MAYAGARFVF SLVDAMNGKE GVVECSFVKS QETECTYFST PLLLGKKGIE
310 320 330
KNLGIGKVSS FEEKMISDAI PELKASIKKG EDFVKTLK
Length:338
Mass (Da):35,503
Last modified:November 25, 2008 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iAAB9F5E5B2FBC8CA
GO
Isoform 2 (identifier: P40926-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     144-185: Missing.

Note: No experimental confirmation available.
Show »
Length:296
Mass (Da):30,875
Checksum:i309B55235F0097F7
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
G3XAL0G3XAL0_HUMAN
Malate dehydrogenase
MDH2 hCG_1818102
231Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
U3KQ63U3KQ63_HUMAN
Malate dehydrogenase, mitochondrial
MDH2
28Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti301K → R in BAG56955 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0477879A → V2 PublicationsCorresponds to variant dbSNP:rs6720Ensembl.1
Natural variantiVAR_07800137G → R in EIEE51; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs782308462EnsemblClinVar.1
Natural variantiVAR_078002133P → L in EIEE51; decreased protein abundance; strong decrease in malate dehydrogenase activity; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs375002796EnsemblClinVar.1
Natural variantiVAR_078003207P → L in EIEE51; decreased protein abundance; strong decrease in malate dehydrogenase activity; severe defects in aerobic respiration, when assayed in a heterologous system. 1 PublicationCorresponds to variant dbSNP:rs1057519566Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_055312144 – 185Missing in isoform 2. 1 PublicationAdd BLAST42

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
AF047470 mRNA Translation: AAC03787.1
AK290779 mRNA Translation: BAF83468.1
AK293460 mRNA Translation: BAG56955.1
AK316587 mRNA Translation: BAG38175.1
AC005077 Genomic DNA No translation available.
AC006330 Genomic DNA No translation available.
CH471220 Genomic DNA Translation: EAW71796.1
BC001917 mRNA Translation: AAH01917.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS5581.1 [P40926-1]
CCDS64691.1 [P40926-2]

NCBI Reference Sequences

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RefSeqi
NP_001269332.1, NM_001282403.1 [P40926-2]
NP_001269333.1, NM_001282404.1
NP_005909.2, NM_005918.3 [P40926-1]

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.520967

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000315758; ENSP00000327070; ENSG00000146701 [P40926-1]
ENST00000432020; ENSP00000408649; ENSG00000146701 [P40926-2]

Database of genes from NCBI RefSeq genomes

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GeneIDi
4191

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:4191

UCSC genome browser

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UCSCi
uc003ueo.5 human [P40926-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>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 (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Wikipedia

Malate dehydrogenase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF047470 mRNA Translation: AAC03787.1
AK290779 mRNA Translation: BAF83468.1
AK293460 mRNA Translation: BAG56955.1
AK316587 mRNA Translation: BAG38175.1
AC005077 Genomic DNA No translation available.
AC006330 Genomic DNA No translation available.
CH471220 Genomic DNA Translation: EAW71796.1
BC001917 mRNA Translation: AAH01917.1
CCDSiCCDS5581.1 [P40926-1]
CCDS64691.1 [P40926-2]
RefSeqiNP_001269332.1, NM_001282403.1 [P40926-2]
NP_001269333.1, NM_001282404.1
NP_005909.2, NM_005918.3 [P40926-1]
UniGeneiHs.520967

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2DFDX-ray1.90A/B/C/D20-338[»]
4WLEX-ray1.90A/B/C/D20-338[»]
4WLFX-ray2.20A/B/C/D20-338[»]
4WLNX-ray2.28A/B/C/D20-338[»]
4WLOX-ray2.50A/B/C/D20-338[»]
4WLUX-ray2.14A/B/C/D20-338[»]
4WLVX-ray2.40A/B/C/D20-338[»]
ProteinModelPortaliP40926
SMRiP40926
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110356, 64 interactors
IntActiP40926, 25 interactors
MINTiP40926
STRINGi9606.ENSP00000327070

Chemistry databases

BindingDBiP40926
ChEMBLiCHEMBL5917
DrugBankiDB04272 Citric Acid
DB00157 NADH

PTM databases

iPTMnetiP40926
PhosphoSitePlusiP40926
SwissPalmiP40926

Polymorphism and mutation databases

BioMutaiMDH2
DMDMi215274114

2D gel databases

DOSAC-COBS-2DPAGEiP40926
REPRODUCTION-2DPAGEiIPI00291006
P40926
UCD-2DPAGEiP40926

Proteomic databases

EPDiP40926
MaxQBiP40926
PaxDbiP40926
PeptideAtlasiP40926
PRIDEiP40926
ProteomicsDBi55386
TopDownProteomicsiP40926-1 [P40926-1]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
4191
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000315758; ENSP00000327070; ENSG00000146701 [P40926-1]
ENST00000432020; ENSP00000408649; ENSG00000146701 [P40926-2]
GeneIDi4191
KEGGihsa:4191
UCSCiuc003ueo.5 human [P40926-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
4191
DisGeNETi4191
EuPathDBiHostDB:ENSG00000146701.11

GeneCards: human genes, protein and diseases

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GeneCardsi
MDH2
HGNCiHGNC:6971 MDH2
HPAiHPA019714
HPA019716
HPA019848
HPA026720
MalaCardsiMDH2
MIMi154100 gene
617339 phenotype
neXtProtiNX_P40926
OpenTargetsiENSG00000146701
Orphaneti29072 Hereditary pheochromocytoma-paraganglioma
PharmGKBiPA30716

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG1494 Eukaryota
COG0039 LUCA
GeneTreeiENSGT00390000016686
HOGENOMiHOG000213792
HOVERGENiHBG001662
InParanoidiP40926
KOiK00026
OMAiQCTPKVE
OrthoDBiEOG091G0AVO
PhylomeDBiP40926
TreeFamiTF300834

Enzyme and pathway databases

BioCyciMetaCyc:HS07366-MONOMER
ReactomeiR-HSA-70263 Gluconeogenesis
R-HSA-71403 Citric acid cycle (TCA cycle)

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
MDH2 human
EvolutionaryTraceiP40926

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
Malate_dehydrogenase_2

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
4191

Protein Ontology

More...
PROi
PR:P40926

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000146701 Expressed in 244 organ(s), highest expression level in vastus lateralis
CleanExiHS_MDH2
ExpressionAtlasiP40926 baseline and differential
GenevisibleiP40926 HS

Family and domain databases

Gene3Di3.90.110.10, 1 hit
InterProiView protein in InterPro
IPR001557 L-lactate/malate_DH
IPR022383 Lactate/malate_DH_C
IPR001236 Lactate/malate_DH_N
IPR015955 Lactate_DH/Glyco_Ohase_4_C
IPR001252 Malate_DH_AS
IPR010097 Malate_DH_type1
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF02866 Ldh_1_C, 1 hit
PF00056 Ldh_1_N, 1 hit
PIRSFiPIRSF000102 Lac_mal_DH, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit
SSF56327 SSF56327, 1 hit
TIGRFAMsiTIGR01772 MDH_euk_gproteo, 1 hit
PROSITEiView protein in PROSITE
PS00068 MDH, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiMDHM_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P40926
Secondary accession number(s): A8K414
, B2RE78, B4DE44, E9PDB2, O43682
<p>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’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1995
Last sequence update: November 25, 2008
Last modified: December 5, 2018
This is version 195 of the entry and version 3 of the sequence. See complete history.
<p>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 (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  2. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  3. SIMILARITY comments
    Index of protein domains and families
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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