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Guidelines

MIRAGE

glycine decarboxylase

Summary

Gene Symbol

GLDC

Aliases

GCSP
NKH
glycine cleavage system protein P
glycine dehydrogenase
nonketotic hyperglycinemia

Organism

Homo sapiens (human)

External Links

NCBI Gene

2731

HGNC

4313

KEGG Gene ID

hsa:2731

PubChem

2731

Alliance of Genome Resources

HGNC:4313

JoGo

GLDC

TogoVar

GLDC

Annotation

Keyword

3D-structure
Acetylation
Disease variant
Mitochondrion
Oxidoreductase
Proteomics identification
Pyridoxal phosphate
Reference proteome
Transit peptide

Proteins

Displaying 1 entry
UniProt	Protein Name
P23378	Glycine cleavage system P protein Glycine decarboxylase Glycine dehydrogenase (aminomethyl-transferring)

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 10 in total

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GO Term	Evidence Code	PMID
amino acid metabolic process	IEA
glycine metabolic process	IEA
glycine catabolic process	TAS
glycine catabolic process	IDA
glycine catabolic process	IEA

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

catabolic process [inference]

amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

small molecule catabolic process [inference]

carboxylic acid catabolic process [inference]

proteinogenic amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

primary metabolic process [inference]

amino acid metabolic process

amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

proteinogenic amino acid metabolic process [inference]

glycine metabolic process

glycine catabolic process

glycine decarboxylation via glycine cleavage system

proteinogenic amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

small molecule metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

carboxylic acid catabolic process [inference]

proteinogenic amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

proteinogenic amino acid metabolic process [inference]

glycine metabolic process

glycine catabolic process

glycine decarboxylation via glycine cleavage system

proteinogenic amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

small molecule catabolic process [inference]

carboxylic acid catabolic process [inference]

proteinogenic amino acid catabolic process [inference]

glycine catabolic process

glycine decarboxylation via glycine cleavage system

response to stimulus [inference]

response to chemical [inference]

response to lipid [inference]

response to fatty acid [inference]

response to lipoic acid

response to peptide [inference]

response to cytokine [inference]

cellular response to cytokine stimulus [inference]

cellular response to leukemia inhibitory factor

response to leukemia inhibitory factor [inference]

cellular response to leukemia inhibitory factor

response to nitrogen compound [inference]

response to amine [inference]

response to methylamine

response to oxygen-containing compound [inference]

response to fatty acid [inference]

response to lipoic acid

Displaying entries **6 - 8** of 8 in total

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GO Term	Evidence Code	PMID
mitochondrial matrix	TAS
plasma membrane
glycine cleavage complex	NAS

GO Hierarchy

cellular component

protein-containing complex [inference]

catalytic complex [inference]

oxidoreductase complex [inference]

glycine cleavage complex

transferase complex [inference]

glycine cleavage complex

cellular anatomical structure [inference]

nucleoplasm

membrane [inference]

plasma membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying entries **1 - 5** of 11 in total

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GO Term	Evidence Code	PMID
glycine dehydrogenase (decarboxylating) activity	EXP
glycine dehydrogenase (decarboxylating) activity	TAS
glycine dehydrogenase (decarboxylating) activity	IDA
glycine dehydrogenase (decarboxylating) activity	IBA
glycine dehydrogenase (decarboxylating) activity	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

oxidoreductase activity, acting on the CH-NH2 group of donors [inference]

oxidoreductase activity, acting on the CH-NH2 group of donors, disulfide as acceptor [inference]

glycine dehydrogenase (decarboxylating) activity

lyase activity

binding [inference]

protein binding [inference]

identical protein binding [inference]

protein homodimerization activity

protein dimerization activity [inference]

protein homodimerization activity

amino acid binding [inference]

glycine binding

small molecule binding [inference]

vitamin binding [inference]

vitamin B6 binding [inference]

ion binding [inference]

anion binding [inference]

pyridoxal phosphate binding

carboxylic acid binding [inference]

glycine binding

cation binding [inference]

organic acid binding [inference]

carboxylic acid binding [inference]

glycine binding

heterocyclic compound binding [inference]

vitamin B6 binding [inference]

electron transfer activity

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000178445

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K00281

Name

glycine cleavage system P protein (glycine dehydrogenase) [EC:1.4.4.2]

References

Rhea

H⁺ + glycine

CO₂

Enzyme

1.4.4.2

PMID

Disease

Disease Ontology

Displaying **all 2** entries
DO ID	Disease Name	Source
DOID:0070616	glycine encephalopathy 1	Alliance of Genome Resources
DOID:9268	glycine encephalopathy	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **1 - 10** of 20 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000711	Restlessness
HP:0000718	Aggressive behavior
HP:0000737	Irritability
HP:0000752	Hyperactivity
HP:0001249	Intellectual disability
HP:0001250	Seizure
HP:0001252	Hypotonia
HP:0001254	Lethargy
HP:0001265	Hyporeflexia

Displaying 1 entry
Disease ID	Disease Name
OMIM:605899	glycine encephalopathy 1

PubChem Disease

GHR Health Conditions

Nonketotic hyperglycinemia

MedGen Diseases

Glycine encephalopathy 1

OMIM Phenotypes

GLYCINE ENCEPHALOPATHY 1; GCE1

KEGG Diseases

Nonketotic hyperglycinemia

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

Displaying 1 entry
Species	Gene ID	Alliance of Genome Resources
Mus musculus	104174	MGI:1341155

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International Collaboration

GlyCosmos is a member of the GlySpace Alliance together with GlyGen and Glycomics@ExPASy.

Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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GlyCosmos Portal v4.5.0

Last updated: April 6, 2026