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MIRAGE

procollagen-lysine,2-oxoglutarate 5-dioxygenase 3

Summary

Gene Symbol

PLOD3

Aliases

LH3
Multifunctional procollagen lysine hydroxylase and glycosyltransferase LH3
lysyl hydroxlase 3

Organism

Homo sapiens (human)

External Links

NCBI Gene

8985

HGNC

9083

KEGG Gene ID

hsa:8985

PubChem

8985

Alliance of Genome Resources

HGNC:9083

JoGo

PLOD3

TogoVar

PLOD3

Annotation

Keyword

3D-structure
Dioxygenase
Disease variant
Disulfide bond
Endoplasmic reticulum
Glycoprotein
Glycosyltransferase
Iron
Manganese
Membrane
Metal-binding
Multifunctional enzyme
Proteomics identification
Reference proteome
Secreted
Signal
Vitamin C

Proteins

Displaying 1 entry
UniProt	Protein Name
O60568

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 14 in total

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GO Term	Evidence Code	PMID
in utero embryonic development
endothelial cell morphogenesis
neural tube development
collagen fibril organization	TAS
collagen fibril organization	IBA

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

biosynthetic process [inference]

macromolecule biosynthetic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

collagen biosynthetic process

carbohydrate derivative biosynthetic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

collagen metabolic process [inference]

collagen biosynthetic process

macromolecule metabolic process [inference]

macromolecule biosynthetic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

protein metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

primary metabolic process [inference]

protein metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

carbohydrate derivative metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

carbohydrate derivative biosynthetic process [inference]

glycoprotein biosynthetic process [inference]

protein O-linked glycosylation [inference]

protein O-linked glycosylation via galactose

cellular component organization or biogenesis [inference]

cellular component organization [inference]

cellular component assembly [inference]

extracellular matrix assembly [inference]

basement membrane assembly

extracellular structure organization [inference]

extracellular matrix organization [inference]

collagen fibril organization

basement membrane organization [inference]

basement membrane assembly

extracellular matrix assembly [inference]

basement membrane assembly

external encapsulating structure organization [inference]

extracellular matrix organization [inference]

collagen fibril organization

basement membrane organization [inference]

basement membrane assembly

extracellular matrix assembly [inference]

basement membrane assembly

multicellular organismal process [inference]

system process [inference]

circulatory system process [inference]

vascular process in circulatory system [inference]

blood vessel diameter maintenance [inference]

vasodilation

multicellular organism development [inference]

embryo development [inference]

embryo development ending in birth or egg hatching [inference]

chordate embryonic development [inference]

in utero embryonic development

developmental process [inference]

anatomical structure morphogenesis [inference]

cell morphogenesis [inference]

epithelial cell morphogenesis [inference]

endothelial cell morphogenesis

animal organ morphogenesis [inference]

lung morphogenesis

tissue morphogenesis [inference]

morphogenesis of an epithelium [inference]

epidermis morphogenesis

anatomical structure development [inference]

multicellular organism development [inference]

embryo development [inference]

embryo development ending in birth or egg hatching [inference]

chordate embryonic development [inference]

in utero embryonic development

tissue development [inference]

epithelium development [inference]

neural tube development

tube development [inference]

neural tube development

localization [inference]

macromolecule localization [inference]

intracellular protein localization

biological regulation [inference]

regulation of biological quality [inference]

regulation of anatomical structure size [inference]

regulation of tube size [inference]

regulation of tube diameter [inference]

blood vessel diameter maintenance [inference]

vasodilation

Displaying entries **6 - 10** of 21 in total

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GO Term	Evidence Code	PMID
endoplasmic reticulum	IDA
endoplasmic reticulum	IBA
endoplasmic reticulum	IEA
endoplasmic reticulum lumen	EXP
endoplasmic reticulum lumen	IEA

GO Hierarchy

cellular component

cellular anatomical structure [inference]

extracellular region

membrane [inference]

organelle membrane [inference]

endoplasmic reticulum membrane

external encapsulating structure [inference]

extracellular matrix

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

endoplasmic reticulum lumen

organelle subcompartment [inference]

Golgi apparatus subcompartment [inference]

trans-Golgi network

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

endoplasmic reticulum

rough endoplasmic reticulum

Golgi apparatus

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

endoplasmic reticulum

rough endoplasmic reticulum

Golgi apparatus

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

Displaying entries **1 - 5** of 20 in total

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GO Term	Evidence Code	PMID
iron ion binding	EXP
iron ion binding	IEA
protein binding	IPI
procollagen-lysine 5-dioxygenase activity	EXP
procollagen-lysine 5-dioxygenase activity	IDA

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen

2-oxoglutarate-dependent dioxygenase activity [inference]

procollagen-lysine 5-dioxygenase activity

dioxygenase activity [inference]

2-oxoglutarate-dependent dioxygenase activity [inference]

procollagen-lysine 5-dioxygenase activity

transferase activity [inference]

glycosyltransferase activity [inference]

UDP-glycosyltransferase activity [inference]

UDP-galactosyltransferase activity [inference]

procollagen galactosyltransferase activity

hexosyltransferase activity [inference]

galactosyltransferase activity [inference]

UDP-galactosyltransferase activity [inference]

procollagen galactosyltransferase activity

glucosyltransferase activity [inference]

procollagen glucosyltransferase activity

catalytic activity, acting on a protein [inference]

binding [inference]

protein binding

carbohydrate binding [inference]

monosaccharide binding [inference]

L-ascorbic acid binding

small molecule binding

vitamin binding [inference]

L-ascorbic acid binding

ion binding [inference]

anion binding [inference]

carboxylic acid binding [inference]

L-ascorbic acid binding

cation binding [inference]

metal ion binding

transition metal ion binding [inference]

iron ion binding

organic acid binding [inference]

carboxylic acid binding [inference]

L-ascorbic acid binding

monosaccharide binding [inference]

L-ascorbic acid binding

heterocyclic compound binding [inference]

L-ascorbic acid binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

procollagen-lysine 2-oxoglutarate 5-dioxygenase 2

Functional Category

E: Amino acid transport and metabolism
O: Posttranslational modification, protein turnover, chaperones
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying **all 7** entries
Gene Ontology
L-ascorbic acid binding
dioxygenase activity
iron ion binding
metal ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen
oxidoreductase activity
procollagen-lysine 5-dioxygenase activity

Displaying **all 5** entries
InterPro
Isopenicillin N synthase-like, Fe(2+) 2OG dioxygenase domain
Nucleotide-diphospho-sugar transferases
Oxoglutarate/iron-dependent dioxygenase
Procollagen-lysine 5-dioxygenase, conserved site
Prolyl 4-hydroxylase, alpha subunit

Human Protein Atlas

ENSG00000106397

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

K13646

Name

lysyl hydroxylase/galactosyltransferase/glucosyltransferase [EC:1.14.11.4 2.4.1.50 2.4.1.66]

References

Disease

The Human Phenotype Ontology

Displaying entries **1 - 10** of 39 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000164	Abnormality of the dentition
HP:0000272	Malar flattening
HP:0000307	Pointed chin
HP:0000369	Low-set ears
HP:0000407	Sensorineural hearing impairment
HP:0000463	Anteverted nares
HP:0000518	Cataract
HP:0000545	Myopia
HP:0000586	Shallow orbits

Displaying 1 entry
Disease ID	Disease Name
OMIM:612394	bone fragility with contractures, arterial rupture, and deafness

PubChem Disease

MedGen Diseases

Bone fragility with contractures, arterial rupture, and deafness

OMIM Phenotypes

BONE FRAGILITY WITH CONTRACTURES, ARTERIAL RUPTURE, AND DEAFNESS; BCARD

KEGG Diseases

Lysyl hydroxylase 3 deficiency

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

Ortholog

Displaying **all 6** entries
Species	Gene ID	Alliance of Genome Resources
Mus musculus	26433	MGI:1347008
Rattus norvegicus	288583	RGD:631339
Drosophila melanogaster	39265	FB:FBgn0036147
Danio rerio	556077	ZFIN:ZDB-GENE-021031-4
Xenopus laevis	380138	Xenbase:XB-GENE-5916727
Xenopus tropicalis	734090	Xenbase:XB-GENE-5916631

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Supported by JST NBDC Grant Number JPMJND2204

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

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Last updated: April 6, 2026