Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.

UniProtKB - P23378 (GCSP_HUMAN)

Entry version 185 (10 Feb 2021)
Sequence version 2 (05 May 2009)
Previous versions | rss
Add a publicationFeedback
Protein

Glycine dehydrogenase (decarboxylating), mitochondrial

Gene

GLDC

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

The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO2 is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH).3 Publications

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">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 provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

pyridoxal 5'-phosphateBy similarity

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> 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

Stimulated by lipoic acid. Inhibited in presence of methylamine (By similarity).By similarity

<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

Complete GO annotation on QuickGO ...

GO - Biological processi

Complete GO annotation on QuickGO ...

<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
LigandPyridoxal phosphate

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...BioCyci		MetaCyc:HS00622-MONOMER

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P23378

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-6783984, Glycine degradation

SABIO-RK: Biochemical Reaction Kinetics Database

More...SABIO-RKi		P23378

<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%5Fand%5Ftaxonomy%5Fsection">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:
Glycine dehydrogenase (decarboxylating), mitochondrial1 Publication (EC:1.4.4.23 Publications)
Alternative name(s):
Glycine cleavage system P protein
Glycine decarboxylase2 Publications
Glycine dehydrogenase (aminomethyl-transferring)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">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:GLDCImported
Synonyms:GCSPImported
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">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%5Fand%5Ftaxonomy%5Fsection">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%5Fand%5Ftaxonomy%5Fsection">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 lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">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%5Fmanual">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 9

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:4313, GLDC

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		238300, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P23378

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000178445.8

<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 & Seán O’Donoghue; 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

Non-ketotic hyperglycinemia (NKH)6 Publications
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionAutosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Related information in OMIM
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_078776146T → K in NKH; loss of glycine catabolic process; loss of expression. 1 PublicationCorresponds to variant dbSNP:rs376578742EnsemblClinVar.1
Natural variantiVAR_078777173L → P in NKH; loss of glycine catabolic process; decreased abundance. 1 Publication1
Natural variantiVAR_078778267P → A in NKH; decreased glycine catabolic process; changed localization to the mitochondria; also expressed diffusely throughout the cytosol; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs1554648117EnsemblClinVar.1
Natural variantiVAR_016849283A → P in NKH. 1 PublicationCorresponds to variant dbSNP:rs386833589EnsemblClinVar.1
Natural variantiVAR_078779362R → C in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs10975674EnsemblClinVar.1
Natural variantiVAR_078780373R → W in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs150171524EnsemblClinVar.1
Natural variantiVAR_078781376K → E in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs774093619Ensembl.1
Natural variantiVAR_078782461R → W in NKH; decreased glycine catabolic process; changed localization to the mitochondria; also expressed diffusely throughout the cytosol; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs761957837EnsemblClinVar.1
Natural variantiVAR_016851515R → S in NKH. 1 PublicationCorresponds to variant dbSNP:rs121964976EnsemblClinVar.1
Natural variantiVAR_078783548L → P in NKH; decreased GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_004979564S → I in NKH; common mutation in Finland. 1 PublicationCorresponds to variant dbSNP:rs121964974EnsemblClinVar.1
Natural variantiVAR_078784580H → Y in NKH; loss of glycine catabolic process; loss of expression. 1 Publication1
Natural variantiVAR_078785581P → R in NKH; loss of glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs772871471EnsemblClinVar.1
Natural variantiVAR_078786624A → D in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_009939756Missing in NKH. 1 Publication1
Natural variantiVAR_078787763G → D in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 PublicationCorresponds to variant dbSNP:rs1374110692Ensembl.1
Natural variantiVAR_078788768G → E in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_078789790R → W in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs386833556EnsemblClinVar.1
Natural variantiVAR_079313839Y → C in NKH. 1 Publication1
Natural variantiVAR_078790866D → H in NKH; decreased glycine catabolic process; decreased abundance. 1 Publication1
Natural variantiVAR_078791905V → G in NKH; decreased GCS P-protein glycine exchange activity; no effect on abundance. 1 PublicationCorresponds to variant dbSNP:rs188269735EnsemblClinVar.1
Natural variantiVAR_078792933I → T in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs758029533EnsemblClinVar.1
Natural variantiVAR_078793994G → R in NKH; loss of glycine catabolic process; loss of expression. 1 PublicationCorresponds to variant dbSNP:rs1406713104EnsemblClinVar.1

Keywords - Diseasei

Disease variant

Organism-specific databases

DisGeNET

More...DisGeNETi		2731

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...GeneReviewsi		GLDC

MalaCards human disease database

More...MalaCardsi		GLDC
MIMi605899, phenotype

Open Targets

More...OpenTargetsi		ENSG00000178445

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

More...Orphaneti		289863, Atypical glycine encephalopathy
289860, Infantile glycine encephalopathy
289857, Neonatal glycine encephalopathy

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA28716

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P23378, Tbio

Chemistry databases

Drug and drug target database

More...DrugBanki		DB00145, Glycine
DB00114, Pyridoxal phosphate

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		GLDC

Domain mapping of disease mutations (DMDM)

More...DMDMi		229462870

<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 – 35MitochondrionSequence analysisAdd BLAST35
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000001074036 – 1020Glycine dehydrogenase (decarboxylating), mitochondrialAdd BLAST985

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<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 residuei447N6-acetyllysineBy similarity1
Modified residuei514N6-acetyllysineBy similarity1
Modified residuei648N6-acetyllysineBy similarity1
Modified residuei664N6-acetyllysineBy similarity1
Modified residuei754N6-(pyridoxal phosphate)lysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P23378

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P23378

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P23378

MaxQB - The MaxQuant DataBase

More...MaxQBi		P23378

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

More...PaxDbi		P23378

PeptideAtlas

More...PeptideAtlasi		P23378

PRoteomics IDEntifications database

More...PRIDEi		P23378

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		54084

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P23378

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

More...PhosphoSitePlusi		P23378

<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		ENSG00000178445, Expressed in placenta and 179 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P23378, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P23378, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000178445, Group enriched (kidney, liver, placenta)

<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%5Fsection">'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%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer (By similarity).

Interacts with GCSH (By similarity). The glycine cleavage system is composed of four proteins: P (GLDC), T (GCST), L (DLD) and H (GCSH).

By similarity

GO - Molecular functioni

Complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		108993, 23 interactors

Protein interaction database and analysis system

More...IntActi		P23378, 17 interactors

Molecular INTeraction database

More...MINTi		P23378

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000370737

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P23378, protein

<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

11020
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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

More...SMRi		P23378

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

<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 GcvP family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG2040, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00390000017970

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		CLU_004620_3_2_1

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P23378

Identification of Orthologs from Complete Genome Data

More...OMAi		CVPMSEY

Database of Orthologous Groups

More...OrthoDBi		390348at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P23378

TreeFam database of animal gene trees

More...TreeFami		TF300678

Family and domain databases

Conserved Domains Database

More...CDDi		cd00613, GDC-P, 2 hits

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.40.640.10, 2 hits
3.90.1150.10, 1 hit

HAMAP database of protein families

More...HAMAPi		MF_00711, GcvP, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR001597, ArAA_b-elim_lyase/Thr_aldolase
IPR003437, GcvP
IPR020581, GDC_P
IPR015424, PyrdxlP-dep_Trfase
IPR015422, PyrdxlP-dep_Trfase_dom1
IPR015421, PyrdxlP-dep_Trfase_major

The PANTHER Classification System

More...PANTHERi		PTHR11773, PTHR11773, 1 hit

Pfam protein domain database

More...Pfami		View protein in Pfam
PF01212, Beta_elim_lyase, 1 hit
PF02347, GDC-P, 1 hit

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF53383, SSF53383, 2 hits

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR00461, gcvP, 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%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">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%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">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 has 1 described isoform and 9 potential isoforms that are computationally mapped.Show allAlign All

P23378-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MQSCARAWGL RLGRGVGGGR RLAGGSGPCW APRSRDSSSG GGDSAAAGAS 
        60         70         80         90        100
RLLERLLPRH DDFARRHIGP GDKDQREMLQ TLGLASIDEL IEKTVPANIR 
       110        120        130        140        150
LKRPLKMEDP VCENEILATL HAISSKNQIW RSYIGMGYYN CSVPQTILRN 
       160        170        180        190        200
LLENSGWITQ YTPYQPEVSQ GRLESLLNYQ TMVCDITGLD MANASLLDEG 
       210        220        230        240        250
TAAAEALQLC YRHNKRRKFL VDPRCHPQTI AVVQTRAKYT GVLTELKLPC 
       260        270        280        290        300
EMDFSGKDVS GVLFQYPDTE GKVEDFTELV ERAHQSGSLA CCATDLLALC 
       310        320        330        340        350
ILRPPGEFGV DIALGSSQRF GVPLGYGGPH AAFFAVRESL VRMMPGRMVG 
       360        370        380        390        400
VTRDATGKEV YRLALQTREQ HIRRDKATSN ICTAQALLAN MAAMFAIYHG 
       410        420        430        440        450
SHGLEHIARR VHNATLILSE GLKRAGHQLQ HDLFFDTLKI QCGCSVKEVL 
       460        470        480        490        500
GRAAQRQINF RLFEDGTLGI SLDETVNEKD LDDLLWIFGC ESSAELVAES 
       510        520        530        540        550
MGEECRGIPG SVFKRTSPFL THQVFNSYHS ETNIVRYMKK LENKDISLVH 
       560        570        580        590        600
SMIPLGSCTM KLNSSSELAP ITWKEFANIH PFVPLDQAQG YQQLFRELEK 
       610        620        630        640        650
DLCELTGYDQ VCFQPNSGAQ GEYAGLATIR AYLNQKGEGH RTVCLIPKSA 
       660        670        680        690        700
HGTNPASAHM AGMKIQPVEV DKYGNIDAVH LKAMVDKHKE NLAAIMITYP 
       710        720        730        740        750
STNGVFEENI SDVCDLIHQH GGQVYLDGAN MNAQVGICRP GDFGSDVSHL 
       760        770        780        790        800
NLHKTFCIPH GGGGPGMGPI GVKKHLAPFL PNHPVISLKR NEDACPVGTV 
       810        820        830        840        850
SAAPWGSSSI LPISWAYIKM MGGKGLKQAT ETAILNANYM AKRLETHYRI 
       860        870        880        890        900
LFRGARGYVG HEFILDTRPF KKSANIEAVD VAKRLQDYGF HAPTMSWPVA 
       910        920        930        940        950
GTLMVEPTES EDKAELDRFC DAMISIRQEI ADIEEGRIDP RVNPLKMSPH 
       960        970        980        990       1000
SLTCVTSSHW DRPYSREVAA FPLPFVKPEN KFWPTIARID DIYGDQHLVC 
      1010       1020
TCPPMEVYES PFSEQKRASS
Length:1,020
Mass (Da):112,730
Last modified:May 5, 2009 - v2
<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:i8FAEA7D56BEB17B2
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 9 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A1W2PP74A0A1W2PP74_HUMAN
Glycine dehydrogenase (aminomethyl-...
GLDC
420Annotation 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>
A0A1W2PQV3A0A1W2PQV3_HUMAN
Glycine dehydrogenase (aminomethyl-...
GLDC
388Annotation 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>
A0A1W2PPE1A0A1W2PPE1_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
164Annotation 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>
A0A1W2PPG0A0A1W2PPG0_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
55Annotation 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>
A0A1W2PPH6A0A1W2PPH6_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
140Annotation 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>
A0A1W2PPB1A0A1W2PPB1_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
254Annotation 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>
A0A1W2PQU6A0A1W2PQU6_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
106Annotation 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>
A0A1W2PPK8A0A1W2PPK8_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
103Annotation 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>
A0A1W2PPD7A0A1W2PPD7_HUMAN
Glycine dehydrogenase (decarboxylat...
GLDC
64Annotation 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 conflicti396A → R in AAA36463 (PubMed:1993704).Curated1
Sequence conflicti396A → R in BAA14286 (PubMed:1993704).Curated1
Sequence conflicti441Q → H in AAA36478 (PubMed:1996985).Curated1
Sequence conflicti441Q → H in AAA36463 (PubMed:1993704).Curated1
Sequence conflicti441Q → H in BAA14286 (PubMed:1993704).Curated1
Sequence conflicti608Y → H in AAA36478 (PubMed:1996985).Curated1
Sequence conflicti976V → M in AAA36463 (PubMed:1993704).Curated1
Sequence conflicti976V → M in BAA14286 (PubMed:1993704).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_078776146T → K in NKH; loss of glycine catabolic process; loss of expression. 1 PublicationCorresponds to variant dbSNP:rs376578742EnsemblClinVar.1
Natural variantiVAR_078777173L → P in NKH; loss of glycine catabolic process; decreased abundance. 1 Publication1
Natural variantiVAR_078778267P → A in NKH; decreased glycine catabolic process; changed localization to the mitochondria; also expressed diffusely throughout the cytosol; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs1554648117EnsemblClinVar.1
Natural variantiVAR_016849283A → P in NKH. 1 PublicationCorresponds to variant dbSNP:rs386833589EnsemblClinVar.1
Natural variantiVAR_016850329P → T in one non-ketotic hyperglycinemia patient. 1 PublicationCorresponds to variant dbSNP:rs386833593EnsemblClinVar.1
Natural variantiVAR_078779362R → C in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs10975674EnsemblClinVar.1
Natural variantiVAR_078780373R → W in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs150171524EnsemblClinVar.1
Natural variantiVAR_078781376K → E in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs774093619Ensembl.1
Natural variantiVAR_078782461R → W in NKH; decreased glycine catabolic process; changed localization to the mitochondria; also expressed diffusely throughout the cytosol; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs761957837EnsemblClinVar.1
Natural variantiVAR_016851515R → S in NKH. 1 PublicationCorresponds to variant dbSNP:rs121964976EnsemblClinVar.1
Natural variantiVAR_078783548L → P in NKH; decreased GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_004979564S → I in NKH; common mutation in Finland. 1 PublicationCorresponds to variant dbSNP:rs121964974EnsemblClinVar.1
Natural variantiVAR_078784580H → Y in NKH; loss of glycine catabolic process; loss of expression. 1 Publication1
Natural variantiVAR_078785581P → R in NKH; loss of glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs772871471EnsemblClinVar.1
Natural variantiVAR_078786624A → D in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_009939756Missing in NKH. 1 Publication1
Natural variantiVAR_078787763G → D in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 PublicationCorresponds to variant dbSNP:rs1374110692Ensembl.1
Natural variantiVAR_078788768G → E in NKH; loss of GCS P-protein glycine exchange activity; no effect on abundance. 1 Publication1
Natural variantiVAR_078789790R → W in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs386833556EnsemblClinVar.1
Natural variantiVAR_079313839Y → C in NKH. 1 Publication1
Natural variantiVAR_078790866D → H in NKH; decreased glycine catabolic process; decreased abundance. 1 Publication1
Natural variantiVAR_078791905V → G in NKH; decreased GCS P-protein glycine exchange activity; no effect on abundance. 1 PublicationCorresponds to variant dbSNP:rs188269735EnsemblClinVar.1
Natural variantiVAR_078792933I → T in NKH; decreased glycine catabolic process; decreased abundance. 1 PublicationCorresponds to variant dbSNP:rs758029533EnsemblClinVar.1
Natural variantiVAR_078793994G → R in NKH; loss of glycine catabolic process; loss of expression. 1 PublicationCorresponds to variant dbSNP:rs1406713104EnsemblClinVar.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...DDBJi
Links Updated
M63635 mRNA Translation: AAA36478.1
M64590 mRNA Translation: AAA36463.1
D90239 mRNA Translation: BAA14286.1
AK314156 mRNA Translation: BAG36841.1
AL353718 Genomic DNA No translation available.
AL162411 Genomic DNA No translation available.
CH471071 Genomic DNA Translation: EAW58740.1
BC111993 mRNA Translation: AAI11994.1
BC111995 mRNA Translation: AAI11996.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS34987.1

Protein sequence database of the Protein Information Resource

More...PIRi		JN0124

NCBI Reference Sequences

More...RefSeqi		NP_000161.2, NM_000170.2

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000321612; ENSP00000370737; ENSG00000178445

Database of genes from NCBI RefSeq genomes

More...GeneIDi		2731

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:2731

UCSC genome browser

More...UCSCi		uc003zkc.4, human

<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

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M63635 mRNA Translation: AAA36478.1
M64590 mRNA Translation: AAA36463.1
D90239 mRNA Translation: BAA14286.1
AK314156 mRNA Translation: BAG36841.1
AL353718 Genomic DNA No translation available.
AL162411 Genomic DNA No translation available.
CH471071 Genomic DNA Translation: EAW58740.1
BC111993 mRNA Translation: AAI11994.1
BC111995 mRNA Translation: AAI11996.1
CCDSiCCDS34987.1
PIRiJN0124
RefSeqiNP_000161.2, NM_000170.2

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
6I33X-ray2.30A/B45-1020[»]
6I34X-ray2.10A/B/C/D45-1020[»]
6I35X-ray2.00A/B/C/D45-1020[»]
SMRiP23378
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi108993, 23 interactors
IntActiP23378, 17 interactors
MINTiP23378
STRINGi9606.ENSP00000370737

Chemistry databases

DrugBankiDB00145, Glycine
DB00114, Pyridoxal phosphate

PTM databases

iPTMnetiP23378
PhosphoSitePlusiP23378

Genetic variation databases

BioMutaiGLDC
DMDMi229462870

Proteomic databases

EPDiP23378
jPOSTiP23378
MassIVEiP23378
MaxQBiP23378
PaxDbiP23378
PeptideAtlasiP23378
PRIDEiP23378
ProteomicsDBi54084

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		9715, 190 antibodies

Genome annotation databases

EnsembliENST00000321612; ENSP00000370737; ENSG00000178445
GeneIDi2731
KEGGihsa:2731
UCSCiuc003zkc.4, human

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		2731
DisGeNETi2731

GeneCards: human genes, protein and diseases

More...GeneCardsi		GLDC
GeneReviewsiGLDC
HGNCiHGNC:4313, GLDC
HPAiENSG00000178445, Group enriched (kidney, liver, placenta)
MalaCardsiGLDC
MIMi238300, gene
605899, phenotype
neXtProtiNX_P23378
OpenTargetsiENSG00000178445
Orphaneti289863, Atypical glycine encephalopathy
289860, Infantile glycine encephalopathy
289857, Neonatal glycine encephalopathy
PharmGKBiPA28716
VEuPathDBiHostDB:ENSG00000178445.8

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG2040, Eukaryota
GeneTreeiENSGT00390000017970
HOGENOMiCLU_004620_3_2_1
InParanoidiP23378
OMAiCVPMSEY
OrthoDBi390348at2759
PhylomeDBiP23378
TreeFamiTF300678

Enzyme and pathway databases

BioCyciMetaCyc:HS00622-MONOMER
PathwayCommonsiP23378
ReactomeiR-HSA-6783984, Glycine degradation
SABIO-RKiP23378

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		2731, 5 hits in 875 CRISPR screens

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

More...ChiTaRSi		GLDC, human

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Glycine_dehydrogenase_(decarboxylating)

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

More...GenomeRNAii		2731
PharosiP23378, Tbio

Protein Ontology

More...PROi		PR:P23378
RNActiP23378, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000178445, Expressed in placenta and 179 other tissues
ExpressionAtlasiP23378, baseline and differential
GenevisibleiP23378, HS

Family and domain databases

CDDicd00613, GDC-P, 2 hits
Gene3Di3.40.640.10, 2 hits
3.90.1150.10, 1 hit
HAMAPiMF_00711, GcvP, 1 hit
InterProiView protein in InterPro
IPR001597, ArAA_b-elim_lyase/Thr_aldolase
IPR003437, GcvP
IPR020581, GDC_P
IPR015424, PyrdxlP-dep_Trfase
IPR015422, PyrdxlP-dep_Trfase_dom1
IPR015421, PyrdxlP-dep_Trfase_major
PANTHERiPTHR11773, PTHR11773, 1 hit
PfamiView protein in Pfam
PF01212, Beta_elim_lyase, 1 hit
PF02347, GDC-P, 1 hit
SUPFAMiSSF53383, SSF53383, 2 hits
TIGRFAMsiTIGR00461, gcvP, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...ProtoNeti		Search...

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		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 nameiGCSP_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: P23378
Secondary accession number(s): Q2M2F8
<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%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1991
Last sequence update: May 5, 2009
Last modified: February 10, 2021
This is version 185 of the entry and version 2 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, Reference proteome

Documents

  1. Human chromosome 9
    Human chromosome 9: entries, gene names and cross-references to MIM
  2. Human variants curated from literature reports
    Index of human variants curated from literature reports
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. SIMILARITY comments
    Index of protein domains and families
  6. Human entries with genetic variants
    List of human entries with genetic variants
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again