GlyCosmos Portal

Annotation

Keyword

3D-structure
Alternative splicing
Disease variant
Lyase
Mitochondrion
Reference proteome
Schiff base
Transit peptide

Proteins

Displaying 1 entry
UniProt	Protein Name
Q86XE5	Dihydrodipicolinate synthase-like Probable 2-keto-4-hydroxyglutarate aldolase Protein 569272

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
glyoxylate metabolic process	IDA	21998747
pyruvate biosynthetic process	IDA	21998747
N-acetylneuraminate catabolic process	IBA
glyoxylate catabolic process	IBA IMP	20797690
oxalate metabolic process	IMP	21896830

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

oxalate metabolic process

amide metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

organonitrogen compound metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

aldehyde catabolic process [inference]

glyoxylate catabolic process

heterocycle catabolic process [inference]

4-hydroxyproline catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

organic substance catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

aldehyde catabolic process [inference]

glyoxylate catabolic process

carbohydrate derivative catabolic process [inference]

amino sugar catabolic process [inference]

N-acetylneuraminate catabolic process

organic cyclic compound catabolic process [inference]

4-hydroxyproline catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

biosynthetic process [inference]

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic substance biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

cellular metabolic process [inference]

cellular aldehyde metabolic process [inference]

aldehyde catabolic process [inference]

glyoxylate catabolic process

glyoxylate metabolic process

glyoxylate catabolic process

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

monocarboxylic acid metabolic process [inference]

pyruvate metabolic process [inference]

pyruvate biosynthetic process

glyoxylate metabolic process

glyoxylate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

dicarboxylic acid metabolic process [inference]

oxalate metabolic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

aldehyde catabolic process [inference]

glyoxylate catabolic process

heterocycle catabolic process [inference]

4-hydroxyproline catabolic process

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

heterocycle metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

heterocycle catabolic process [inference]

4-hydroxyproline catabolic process

primary metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

monocarboxylic acid metabolic process [inference]

pyruvate metabolic process [inference]

pyruvate biosynthetic process

glyoxylate metabolic process

glyoxylate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

dicarboxylic acid metabolic process [inference]

oxalate metabolic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic substance metabolic process [inference]

cellular aldehyde metabolic process [inference]

aldehyde catabolic process [inference]

glyoxylate catabolic process

glyoxylate metabolic process

glyoxylate catabolic process

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

monocarboxylic acid metabolic process [inference]

pyruvate metabolic process [inference]

pyruvate biosynthetic process

glyoxylate metabolic process

glyoxylate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

dicarboxylic acid metabolic process [inference]

oxalate metabolic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

carbohydrate derivative metabolic process [inference]

amino sugar metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino sugar catabolic process [inference]

N-acetylneuraminate catabolic process

carbohydrate derivative catabolic process [inference]

amino sugar catabolic process [inference]

N-acetylneuraminate catabolic process

organic cyclic compound metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

organic cyclic compound catabolic process [inference]

4-hydroxyproline catabolic process

organonitrogen compound metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

organic substance catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

aldehyde catabolic process [inference]

glyoxylate catabolic process

carbohydrate derivative catabolic process [inference]

amino sugar catabolic process [inference]

N-acetylneuraminate catabolic process

organic cyclic compound catabolic process [inference]

4-hydroxyproline catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

organic substance biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

cellular process [inference]

cellular metabolic process [inference]

cellular aldehyde metabolic process [inference]

aldehyde catabolic process [inference]

glyoxylate catabolic process

glyoxylate metabolic process

glyoxylate catabolic process

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

N-acetylneuraminate metabolic process [inference]

N-acetylneuraminate catabolic process

amino acid metabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

monocarboxylic acid metabolic process [inference]

pyruvate metabolic process [inference]

pyruvate biosynthetic process

glyoxylate metabolic process

glyoxylate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

dicarboxylic acid metabolic process [inference]

oxalate metabolic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

cellular modified amino acid catabolic process [inference]

4-hydroxyproline catabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

4-hydroxyproline catabolic process

N-acetylneuraminate catabolic process

monocarboxylic acid catabolic process [inference]

glyoxylate catabolic process

alpha-amino acid catabolic process [inference]

4-hydroxyproline catabolic process

aldehyde catabolic process [inference]

glyoxylate catabolic process

heterocycle catabolic process [inference]

4-hydroxyproline catabolic process

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

pyruvate biosynthetic process

heterocycle metabolic process [inference]

4-hydroxyproline metabolic process [inference]

4-hydroxyproline catabolic process

heterocycle catabolic process [inference]

4-hydroxyproline catabolic process

Displaying **all 2** entries
GO Term	Evidence Code	PMID
mitochondrial matrix
mitochondrion	IBA ISS	21998747

GO Hierarchy

cellular component

cellular anatomical entity [inference]

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 **all 5** entries
GO Term	Evidence Code	PMID
(4S)-4-hydroxy-2-oxoglutarate aldolase activity	IEA
protein homodimerization activity	IDA	21998747
N-acetylneuraminate lyase activity	IBA
4-hydroxy-2-oxoglutarate aldolase activity	IBA IDA ISS	21998747
protein binding	IPI	27499296 32296183

GO Hierarchy

molecular function

catalytic activity [inference]

lyase activity [inference]

carbon-carbon lyase activity [inference]

aldehyde-lyase activity [inference]

4-hydroxy-2-oxoglutarate aldolase activity

oxo-acid-lyase activity [inference]

binding [inference]

protein binding

identical protein binding [inference]

protein homodimerization activity

protein dimerization activity [inference]

protein homodimerization activity

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000241935

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

K18123

Name

4-hydroxy-2-oxoglutarate aldolase [EC:4.1.3.16]

References

21998747

Rhea

RHEA:30687

(4R)-4-hydroxy-2-oxoglutarate

pyruvate + glyoxylate

Enzyme

4.1.3.16

PMID

5780845

RHEA:35639

(4S)-4-hydroxy-2-oxoglutarate

pyruvate + glyoxylate

Enzyme

PMID

Disease

Disease Ontology

Displaying **all 10** entries
DO ID	Disease Name	Source
DOID:0111672	primary hyperoxaluria type 3	Alliance of Genome Resources
DOID:10575	calcium metabolism disease	DisGeNET
DOID:12678	hypercalcemia	DisGeNET
DOID:12679	nephrocalcinosis	DisGeNET
DOID:13580	cholestasis	DisGeNET
DOID:182	calcinosis	DisGeNET
DOID:2485	phosphorus metabolism disease	DisGeNET
DOID:2977	primary hyperoxaluria	DisGeNET DisGeNET DisGeNET Alliance of Genome Resources
DOID:585	nephrolithiasis	DisGeNET
DOID:784	chronic kidney disease	DisGeNET

The Human Phenotype Ontology

Displaying **all 10** entries
HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000121	Nephrocalcinosis
HP:0000790	Hematuria
HP:0003110	Abnormality of urine homeostasis
HP:0003159	Hyperoxaluria
HP:0008672	Calcium oxalate nephrolithiasis
HP:0012211	Abnormal renal physiology
HP:0012531	Pain
HP:0100515	Pollakisuria
HP:0100518	Dysuria

Displaying **all 2** entries
Disease ID	Disease Name
ORPHA:93600	primary hyperoxaluria type 3
OMIM:613616	primary hyperoxaluria type 3

PubChem Disease

GHR Health Conditions

Primary hyperoxaluria

MedGen Diseases

Primary hyperoxaluria type 3

OMIM Phenotypes

HYPEROXALURIA, PRIMARY, TYPE III; HP3

KEGG Diseases

Primary hyperoxaluria

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

Ortholog

Displaying entries **1 - 10** of 94 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Ciona intestinalis	100181036		CIOIN09620
Latimeria chalumnae	102362318		LATCH14406
Danio rerio	405806	ZFIN:ZDB-GENE-040426-2242
Astyanax mexicanus	103023796		ASTMX03611
Ictalurus punctatus	108259603		ICTPU22516
Electrophorus electricus	113577699		ELEEL20830
Salmo salar	106591168		SALSA19329
Salmo salar	106591313		SALSA19329
Salmo trutta	115178813		SALTR67549
Gadus morhua	115540262		GADMO43825

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements