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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

Annotation

Keyword

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

Proteins

Displaying **all 2** entries
UniProt	Protein Name
O60568
Q9UG85

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 13 in total

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GO Term	Evidence Code	PMID
basement membrane assembly	IEA
hydroxylysine biosynthetic process	IEA
protein O-linked glycosylation	IMP	10934207
lung morphogenesis	IEA
protein localization	IEA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid metabolic process [inference]

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

protein metabolic process [inference]

protein modification process [inference]

protein glycosylation [inference]

protein O-linked glycosylation

protein hydroxylation [inference]

peptidyl-lysine hydroxylation

peptidyl-amino acid modification [inference]

peptidyl-lysine modification [inference]

peptidyl-lysine hydroxylation

organonitrogen compound biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

biosynthetic process [inference]

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

organic substance biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

organonitrogen compound biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

collagen metabolic process

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid metabolic process [inference]

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

primary metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

protein metabolic process [inference]

protein modification process [inference]

protein glycosylation [inference]

protein O-linked glycosylation

protein hydroxylation [inference]

peptidyl-lysine hydroxylation

peptidyl-amino acid modification [inference]

peptidyl-lysine modification [inference]

peptidyl-lysine hydroxylation

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

glycosylation [inference]

macromolecule glycosylation [inference]

protein glycosylation [inference]

protein O-linked glycosylation

organic substance metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

macromolecule metabolic process [inference]

protein metabolic process [inference]

protein modification process [inference]

protein glycosylation [inference]

protein O-linked glycosylation

protein hydroxylation [inference]

peptidyl-lysine hydroxylation

peptidyl-amino acid modification [inference]

peptidyl-lysine modification [inference]

peptidyl-lysine hydroxylation

macromolecule modification [inference]

protein modification process [inference]

protein glycosylation [inference]

protein O-linked glycosylation

protein hydroxylation [inference]

peptidyl-lysine hydroxylation

peptidyl-amino acid modification [inference]

peptidyl-lysine modification [inference]

peptidyl-lysine hydroxylation

macromolecule glycosylation [inference]

protein glycosylation [inference]

protein O-linked glycosylation

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid metabolic process [inference]

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

protein metabolic process [inference]

protein modification process [inference]

protein glycosylation [inference]

protein O-linked glycosylation

protein hydroxylation [inference]

peptidyl-lysine hydroxylation

peptidyl-amino acid modification [inference]

peptidyl-lysine modification [inference]

peptidyl-lysine hydroxylation

organonitrogen compound biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

organic substance biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

organonitrogen compound biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular process [inference]

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid metabolic process [inference]

aspartate family amino acid metabolic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular modified amino acid metabolic process [inference]

cellular modified amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

hydroxylysine metabolic process [inference]

hydroxylysine biosynthetic process

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

amino acid biosynthetic process [inference]

alpha-amino acid biosynthetic process [inference]

aspartate family amino acid biosynthetic process [inference]

hydroxylysine biosynthetic process

cellular localization [inference]

cellular macromolecule localization [inference]

protein localization

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

supramolecular fiber organization [inference]

collagen fibril organization

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]

cellular macromolecule localization [inference]

protein localization

cellular localization [inference]

cellular macromolecule localization [inference]

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 **1 - 5** of 9 in total

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GO Term	Evidence Code	PMID
endoplasmic reticulum lumen	IEA
endoplasmic reticulum	IBA IDA TAS	10934207 9724729
trans-Golgi network	IEA
endoplasmic reticulum membrane	TAS
collagen-containing extracellular matrix	IEA

GO Hierarchy

cellular component

cellular anatomical entity [inference]

extracellular space

membrane [inference]

organelle membrane [inference]

endoplasmic reticulum membrane

external encapsulating structure [inference]

extracellular matrix [inference]

collagen-containing 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 8 in total

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GO Term	Evidence Code	PMID
small molecule binding	EXP	30089812
procollagen glucosyltransferase activity	IBA IMP	10934207 18298658
iron ion binding	EXP	30089812
procollagen galactosyltransferase activity	IMP TAS	18298658
metal ion binding	EXP	30089812

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

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

2-oxoglutarate-dependent dioxygenase activity [inference]

peptidyl-lysine 5-dioxygenase activity [inference]

procollagen-lysine 5-dioxygenase activity

dioxygenase activity [inference]

2-oxoglutarate-dependent dioxygenase activity [inference]

peptidyl-lysine 5-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]

peptidyl-lysine 5-dioxygenase activity [inference]

procollagen-lysine 5-dioxygenase activity

binding [inference]

protein binding

carbohydrate binding [inference]

monosaccharide binding [inference]

L-ascorbic acid binding

small molecule binding

vitamin binding [inference]

L-ascorbic acid 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

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 cyclic compound binding [inference]

L-ascorbic acid binding

Gene Ontology information from NCBI Gene and UniProt.

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

Disease Ontology

Displaying entries **1 - 10** of 42 in total

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DO ID	Disease Name	Source
DOID:0050328	congenital hypothyroidism	DisGeNET
DOID:0060058	lymphoma	DisGeNET
DOID:0060249	scoliosis	DisGeNET
DOID:0060735	epidermolysis bullosa simplex Dowling-Meara type	DisGeNET
DOID:0060736	epidermolysis bullosa simplex Ogna type	DisGeNET
DOID:0080001	bone disease	DisGeNET
DOID:0080087	nonsyndromic congenital nail disorder 9	DisGeNET
DOID:0090017	epidermolysis bullosa simplex with muscular dystrophy	DisGeNET
DOID:0111257	gamma-glutamyl transpeptidase deficiency	DisGeNET
DOID:10003	sensorineural hearing loss	DisGeNET

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 entries **1 - 10** of 63 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	174564	WB:WBGene00002497
Drosophila melanogaster	39265	FB:FBgn0036147
Danio rerio	556077	ZFIN:ZDB-GENE-021031-4	DANRE24743
Ictalurus punctatus	108275691		ICTPU07525
Salmo trutta	115183238		SALTR50777
Gadus morhua	115529392		GADMO20708
Oreochromis niloticus	100697420		ORENI49600
Sparus aurata	115589962		SPAAU07491
Xenopus laevis	380138	Xenbase:XB-GENE-5916727
Xenopus laevis	495489	Xenbase:XB-GENE-17339835

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements