List of works - Johanna Myllyharju

A hypodermally expressed prolyl 4-hydroxylase from the filarial nematode Brugia malayi is soluble and active in the absence of protein disulfide isomerase

scientific article published on November 2002

A long hypoxia-inducible factor 3 isoform 2 is a transcription activator that regulates erythropoietin

scientific article published on 25 November 2019

A selective inhibitor and probe of the cellular functions of Jumonji C domain-containing histone demethylases

scientific article

Activation of hypoxia response in endothelial cells contributes to ischemic cardioprotection

scientific article published on 17 June 2013

An endoplasmic reticulum transmembrane prolyl 4-hydroxylase is induced by hypoxia and acts on hypoxia-inducible factor alpha

scientific article published on 27 August 2007

Assembly of stable human type I and III collagen molecules from hydroxylated recombinant chains in the yeast Pichia pastoris. Effect of an engineered C-terminal oligomerization domain foldon

scientific article

Assembly of the elongated collagen prolyl 4-hydroxylase α2β2 heterotetramer around a central α2 dimer.

scientific article published on 16 January 2017

BAMBI is a novel HIF1-dependent modulator of TGFβ-mediated disruption of cell polarity in hypoxia.

scientific article published on 23 April 2018

Blocking Surgically Induced Lysyl Oxidase Activity Reduces the Risk of Lung Metastases.

scientific article

CD146(+) cells are essential for kidney vasculature development.

scientific article published on 18 April 2016

Catalytic properties of the asparaginyl hydroxylase (FIH) in the oxygen sensing pathway are distinct from those of its prolyl 4-hydroxylases

scientific journal article

Characterization of a novel Caenorhabditis elegans prolyl 4-hydroxylase with a unique substrate specificity and restricted expression in the pharynx and excretory duct

scientific article

Characterization of a second Arabidopsis thaliana prolyl 4-hydroxylase with distinct substrate specificity ⬇️

scientific article

Characterization of recombinant human prolyl 3-hydroxylase isoenzyme 2, an enzyme modifying the basement membrane collagen IV

scientific article

Characterization of the human prolyl 4-hydroxylases that modify the hypoxia-inducible factor

scientific journal article

Characterization of three fragments that constitute the monomers of the human lysyl hydroxylase isoenzymes 1-3. The 30-kDa N-terminal fragment is not required for lysyl hydroxylase activity

scientific article

Characterization of two carnation petal prolyl 4 hydroxylases

scientific article published on October 2010

Chlamydomonas reinhardtii has multiple prolyl 4-hydroxylases, one of which is essential for proper cell wall assembly

scientific article published on 12 January 2007

Clinical characterization, genetic mapping and whole-genome sequence analysis of a novel autosomal recessive intellectual disability syndrome

scientific article published on 29 July 2014

Cloning and characterization of a low molecular weight prolyl 4-hydroxylase from Arabidopsis thaliana. Effective hydroxylation of proline-rich, collagen-like, and hypoxia-inducible transcription factor alpha-like peptides

scientific article published on 25 April 2002

Collagen binding specificity of the discoidin domain receptors: binding sites on collagens II and III and molecular determinants for collagen IV recognition by DDR1 ⬇️

scientific article published on October 28, 2010

Collagen prolyl 4-hydroxylase tetramers and dimers show identical decreases in Km values for peptide substrates with increasing chain length: mutation of one of the two catalytic sites in the tetramer inactivates the enzyme by more than half

scientific article published on 25 February 2004

Collagens, modifying enzymes and their mutations in humans, flies and worms

scientific article

Crystallization of the proline-rich-peptide binding domain of human type I collagen prolyl 4-hydroxylase

scientific article published on 25 April 2003

Deficiency of a transmembrane prolyl 4-hydroxylase in the zebrafish leads to basement membrane defects and compromised kidney function

scientific article

Deletion of hypoxia-inducible factor prolyl 4-hydroxylase 2 in FoxD1-lineage mesenchymal cells leads to congenital truncal alopecia

scientific article published on 02 March 2022

Differences in collagen prolyl 4-hydroxylase assembly between two Caenorhabditis nematode species despite high amino acid sequence identity of the enzyme subunits

scientific article published on 30 January 2007

Effect of desferrioxamine and metals on the hydroxylases in the oxygen sensing pathway

scientific article published on 7 June 2005

Egg shell collagen formation in Caenorhabditis elegans involves a novel prolyl 4-hydroxylase expressed in spermatheca and embryos and possessing many unique properties

scientific article published on 12 March 2002

Extracellular matrix and developing growth plate

scientific article published on December 2014

Extracellular matrix genes as hypoxia-inducible targets

scientific article

Fermentation process for tetrameric human collagen prolyl 4-hydroxylase in Escherichia coli: improvement by gene optimisation of the PDI/beta subunit and repeated addition of the inducer anhydrotetracycline

scientific article published on 7 November 2006

HIF Prolyl 4-Hydroxylase-2 Inhibition Improves Glucose and Lipid Metabolism and Protects Against Obesity and Metabolic Dysfunction

scientific article published on 01 May 2014

HIF prolyl 4-hydroxylases and their potential as drug targets

scientific article published on January 2009

HIF-P4H-2 deficiency protects against skeletal muscle ischemia-reperfusion injury.

scientific article published on 10 October 2015

HIF-P4H-2 inhibition enhances intestinal fructose metabolism and induces thermogenesis protecting against NAFLD

scientific article published on 15 April 2020

Hearts of hypoxia-inducible factor prolyl 4-hydroxylase-2 hypomorphic mice show protection against acute ischemia-reperfusion injury

scientific article

High myopia caused by a mutation in LEPREL1, encoding prolyl 3-hydroxylase 2

scientific article

High-level production of human collagen prolyl 4-hydroxylase in Escherichia coli

scientific article

Hypoxia-Inducible Factor Prolyl 4-Hydroxylase-2 Inhibition Protects Against Development of Atherosclerosis.

scientific article published on 4 February 2016

Hypoxia-inducible factor (HIF)-3alpha is subject to extensive alternative splicing in human tissues and cancer cells and is regulated by HIF-1 but not HIF-2.

scientific article published on 21 April 2010

Hypoxia-inducible factor prolyl 4-hydroxylases: common and specific roles

scientific article published on April 2013

Hypoxia-inducible factor prolyl-4-hydroxylase-1 is a convergent point in the reciprocal negative regulation of NF-κB and p53 signaling pathways

scientific article

Hypoxia-inducible factor-1 (HIF-1) but not HIF-2 is essential for hypoxic induction of collagen prolyl 4-hydroxylases in primary newborn mouse epiphyseal growth plate chondrocytes.

scientific article

Identification and characterization of a third human, rat, and mouse collagen prolyl 4-hydroxylase isoenzyme

scientific journal article

Identification and characterization of structural domains of human ERp57: association with calreticulin requires several domains

scientific article published on 19 January 2004

Improved production of human type II procollagen in the yeast Pichia pastoris in shake flasks by a wireless-controlled fed-batch system

scientific article

Influence of the oxidoreductase ER57 on the folding of an antibody fab fragment

scientific article published on 01 August 2004

Inhibition of hypoxia-inducible factor (HIF) hydroxylases by citric acid cycle intermediates: possible links between cell metabolism and stabilization of HIF.

scientific article

Interactions between lysyl oxidases and ADAMTS proteins suggest a novel crosstalk between two extracellular matrix families

scientific article published on 17 May 2018

Kinetic Analysis of HIF Prolyl Hydroxylases

scientific article published in January 2018

Lack of P4H-TM in mice results in age-related retinal and renal alterations.

scientific article published on 27 July 2016

Lack of activity of recombinant HIF prolyl hydroxylases (PHDs) on reported non-HIF substrates

scientific article published on 10 September 2019

Loss of Cln5 leads to altered Gad1 expression and deficits in interneuron development in mice

scientific article published on 01 October 2019

Loss of assembly of the main basement membrane collagen, type IV, but not fibril-forming collagens and embryonic death in collagen prolyl 4-hydroxylase I null mice

scientific journal article

Lysyl hydroxylase 2 is a specific telopeptide hydroxylase, while all three isoenzymes hydroxylate collagenous sequences

scientific article published on 16 January 2007

Lysyl oxidase is essential for normal development and function of the respiratory system and for the integrity of elastic and collagen fibers in various tissues

scientific article

Lysyl oxidase oxidizes cell membrane proteins and enhances the chemotactic response of vascular smooth muscle cells

scientific article published on 27 June 2008

Many amino acid substitutions in a hypoxia-inducible transcription factor (HIF)-1alpha-like peptide cause only minor changes in its hydroxylation by the HIF prolyl 4-hydroxylases: substitution of 3,4-dehydroproline or azetidine-2-carboxylic acid for

scientific article published on 12 October 2004

Missense mutations that cause Bruck syndrome affect enzymatic activity, folding, and oligomerization of lysyl hydroxylase 2

scientific article published on 17 September 2009

Modelling of translation of human protein disulfide isomerase in Escherichia coli-A case study of gene optimisation.

scientific article published in October 2005

Muscle composition is regulated by a Lox-TGFβ feedback loop

scientific article published in March 2015

NMR assignment of the N-terminal domain a of the glycoprotein chaperone ERp57

scientific article published on 01 October 2005

Notch Downregulation and Extramedullary Erythrocytosis in Hypoxia-Inducible Factor Prolyl 4-Hydroxylase 2-Deficient Mice

scientific article

Null mutation in P4h-tm leads to decreased fear and anxiety and increased social behavior in mice

scientific article published on 25 April 2019

Oxygen-dependent ATF-4 stability is mediated by the PHD3 oxygen sensor

scientific article published on 07 August 2007

P4HA1 mutations cause a unique congenital disorder of connective tissue involving tendon, bone, muscle and the eye.

scientific article published on 13 April 2017

PHD1 regulates p53-mediated colorectal cancer chemoresistance.

scientific article published on 19 August 2015

Premature aggregation of type IV collagen and early lethality in lysyl hydroxylase 3 null mice

scholarly article

Proline hydroxylation in collagen supports integrin binding by two distinct mechanisms.

scientific article published on 3 April 2018

Prolyl 4-hydroxlase activity is essential for development and cuticle formation in the human infective parasitic nematode Brugia malayi

scientific article published on 6 December 2012

Prolyl 4-hydroxylases, key enzymes in the synthesis of collagens and regulation of the response to hypoxia, and their roles as treatment targets

scientific article published on January 2008

Prolyl 4-hydroxylases, the key enzymes of collagen biosynthesis ⬇️

scientific article published on March 1, 2003

Prolyl 4-hydroxylation regulates Argonaute 2 stability

scientific article

Prolyl and lysyl hydroxylases in collagen synthesis

scientific article published on 23 September 2020

Prolyl hydroxylase domain 2 reduction enhances skeletal muscle tissue regeneration after soft tissue trauma in mice

scientific article published on 15 May 2020

Recombinant collagen trimers from insect cells and yeast

scientific article published on 01 January 2009

Relevance of posttranslational modifications for the arthritogenicity of type II collagen

scientific article published in March 2004

Role of prolyl hydroxylation in the molecular interactions of collagens

scientific article published on 13 September 2019

Roles of the human hypoxia-inducible factor (HIF)-3α variants in the hypoxia response

scientific article published on 10 April 2011

Sandwich ELISA for quantitative detection of human collagen prolyl 4-hydroxylase

scientific article published on 17 June 2010

Selective expression of nonsecreted triple-helical and secreted single-chain recombinant collagen fragments in the yeast Pichia pastoris

scientific article published on 4 January 2006

Severe Extracellular Matrix Abnormalities and Chondrodysplasia in Mice Lacking Collagen Prolyl 4-Hydroxylase Isoenzyme II in Combination with a Reduced Amount of Isoenzyme I.

scientific article published on 22 May 2015

Structural basis of homo- and heterotrimerization of collagen I

scientific article

Structural enzymology binding studies of the peptide-substrate-binding domain of human collagen prolyl 4-hydroxylase (type-II): High affinity peptides have a PxGP sequence motif

scientific article published on 01 September 2018

Structure of transmembrane prolyl 4-hydroxylase reveals unique organization of EF and dioxygenase domains

scientific article published on 17 December 2020

Systemic inactivation of hypoxia-inducible factor prolyl 4-hydroxylase 2 in mice protects from alcohol-induced fatty liver disease

scientific article published on 19 February 2019

Systemic long-term inactivation of hypoxia-inducible factor prolyl 4-hydroxylase 2 ameliorates aging-induced changes in mice without affecting their life span

scientific article published on 25 February 2020

T-cell activation leads to reduced collagen maturation in atherosclerotic plaques of Apoe(-/-) mice

scientific article

The Crystal Structure of an Algal Prolyl 4-Hydroxylase Complexed with a Proline-rich Peptide Reveals a Novel Buried Tripeptide Binding Motif

scientific article

The Ras signaling inhibitor LOX-PP interacts with Hsp70 and c-Raf to reduce Erk activation and transformed phenotype of breast cancer cells

scientific article

The active site of an algal prolyl 4-hydroxylase has a large structural plasticity

scientific article

The exoskeleton collagens in Caenorhabditis elegans are modified by prolyl 4-hydroxylases with unique combinations of subunits

scientific article

The glucose-sensing transcription factor ChREBP is targeted by proline hydroxylation

scientific article published on 06 October 2020

The length of peptide substrates has a marked effect on hydroxylation by the hypoxia-inducible factor prolyl 4-hydroxylases

scientific article

The peptide-substrate-binding domain of collagen prolyl 4-hydroxylases is a tetratricopeptide repeat domain with functional aromatic residues

scientific article

The peptide-substrate-binding domain of human collagen prolyl 4-hydroxylases. Backbone assignments, secondary structure, and binding of proline-rich peptides

scientific article

The primary substrate binding site in the b' domain of ERp57 is adapted for endoplasmic reticulum lectin association

scientific article published on 10 February 2004

The structural motifs for substrate binding and dimerization of the α subunit of collagen prolyl 4-hydroxylase

scientific article

Three binding sites in protein-disulfide isomerase cooperate in collagen prolyl 4-hydroxylase tetramer assembly.

scientific article

Tissue-specific changes in the hydroxylysine content and cross-links of collagens and alterations in fibril morphology in lysyl hydroxylase 1 knock-out mice

scientific article published on 29 December 2006

Transmembrane Prolyl 4-Hydroxylase Is a Novel Regulator of Calcium Signaling in Astrocytes

scientific article published on 08 December 2020

Transmembrane prolyl 4-hydroxylase is a fourth prolyl 4-hydroxylase regulating EPO production and erythropoiesis

scientific article

Update on hypoxia-inducible factors and hydroxylases in oxygen regulatory pathways: from physiology to therapeutics

scientific article published on 15 March 2017

Wnt5a Deficiency Leads to Anomalies in Ureteric Tree Development, Tubular Epithelial Cell Organization and Basement Membrane Integrity Pointing to a Role in Kidney Collecting Duct Patterning

scientific article

List of works by Johanna Myllyharju

A hypodermally expressed prolyl 4-hydroxylase from the filarial nematode Brugia malayi is soluble and active in the absence of protein disulfide isomerase

A long hypoxia-inducible factor 3 isoform 2 is a transcription activator that regulates erythropoietin

A selective inhibitor and probe of the cellular functions of Jumonji C domain-containing histone demethylases

Activation of hypoxia response in endothelial cells contributes to ischemic cardioprotection

An endoplasmic reticulum transmembrane prolyl 4-hydroxylase is induced by hypoxia and acts on hypoxia-inducible factor alpha

Assembly of stable human type I and III collagen molecules from hydroxylated recombinant chains in the yeast Pichia pastoris. Effect of an engineered C-terminal oligomerization domain foldon

Assembly of the elongated collagen prolyl 4-hydroxylase α2β2 heterotetramer around a central α2 dimer.

BAMBI is a novel HIF1-dependent modulator of TGFβ-mediated disruption of cell polarity in hypoxia.

Blocking Surgically Induced Lysyl Oxidase Activity Reduces the Risk of Lung Metastases.

CD146(+) cells are essential for kidney vasculature development.

Catalytic properties of the asparaginyl hydroxylase (FIH) in the oxygen sensing pathway are distinct from those of its prolyl 4-hydroxylases

Characterization of a novel Caenorhabditis elegans prolyl 4-hydroxylase with a unique substrate specificity and restricted expression in the pharynx and excretory duct

Characterization of a second Arabidopsis thaliana prolyl 4-hydroxylase with distinct substrate specificity ⬇️

Characterization of recombinant human prolyl 3-hydroxylase isoenzyme 2, an enzyme modifying the basement membrane collagen IV

Characterization of the human prolyl 4-hydroxylases that modify the hypoxia-inducible factor

Characterization of three fragments that constitute the monomers of the human lysyl hydroxylase isoenzymes 1-3. The 30-kDa N-terminal fragment is not required for lysyl hydroxylase activity

Characterization of two carnation petal prolyl 4 hydroxylases

Chlamydomonas reinhardtii has multiple prolyl 4-hydroxylases, one of which is essential for proper cell wall assembly

Clinical characterization, genetic mapping and whole-genome sequence analysis of a novel autosomal recessive intellectual disability syndrome

Cloning and characterization of a low molecular weight prolyl 4-hydroxylase from Arabidopsis thaliana. Effective hydroxylation of proline-rich, collagen-like, and hypoxia-inducible transcription factor alpha-like peptides

Collagen binding specificity of the discoidin domain receptors: binding sites on collagens II and III and molecular determinants for collagen IV recognition by DDR1 ⬇️

Collagen prolyl 4-hydroxylase tetramers and dimers show identical decreases in Km values for peptide substrates with increasing chain length: mutation of one of the two catalytic sites in the tetramer inactivates the enzyme by more than half

Collagens, modifying enzymes and their mutations in humans, flies and worms

Crystallization of the proline-rich-peptide binding domain of human type I collagen prolyl 4-hydroxylase

Deficiency of a transmembrane prolyl 4-hydroxylase in the zebrafish leads to basement membrane defects and compromised kidney function

Deletion of hypoxia-inducible factor prolyl 4-hydroxylase 2 in FoxD1-lineage mesenchymal cells leads to congenital truncal alopecia

Differences in collagen prolyl 4-hydroxylase assembly between two Caenorhabditis nematode species despite high amino acid sequence identity of the enzyme subunits

Effect of desferrioxamine and metals on the hydroxylases in the oxygen sensing pathway

Egg shell collagen formation in Caenorhabditis elegans involves a novel prolyl 4-hydroxylase expressed in spermatheca and embryos and possessing many unique properties

Extracellular matrix and developing growth plate

Extracellular matrix genes as hypoxia-inducible targets

Fermentation process for tetrameric human collagen prolyl 4-hydroxylase in Escherichia coli: improvement by gene optimisation of the PDI/beta subunit and repeated addition of the inducer anhydrotetracycline

HIF Prolyl 4-Hydroxylase-2 Inhibition Improves Glucose and Lipid Metabolism and Protects Against Obesity and Metabolic Dysfunction

HIF prolyl 4-hydroxylases and their potential as drug targets

HIF-P4H-2 deficiency protects against skeletal muscle ischemia-reperfusion injury.

HIF-P4H-2 inhibition enhances intestinal fructose metabolism and induces thermogenesis protecting against NAFLD

Hearts of hypoxia-inducible factor prolyl 4-hydroxylase-2 hypomorphic mice show protection against acute ischemia-reperfusion injury

High myopia caused by a mutation in LEPREL1, encoding prolyl 3-hydroxylase 2

High-level production of human collagen prolyl 4-hydroxylase in Escherichia coli

Hypoxia-Inducible Factor Prolyl 4-Hydroxylase-2 Inhibition Protects Against Development of Atherosclerosis.

Hypoxia-inducible factor (HIF)-3alpha is subject to extensive alternative splicing in human tissues and cancer cells and is regulated by HIF-1 but not HIF-2.

Hypoxia-inducible factor prolyl 4-hydroxylases: common and specific roles

Hypoxia-inducible factor prolyl-4-hydroxylase-1 is a convergent point in the reciprocal negative regulation of NF-κB and p53 signaling pathways

Hypoxia-inducible factor-1 (HIF-1) but not HIF-2 is essential for hypoxic induction of collagen prolyl 4-hydroxylases in primary newborn mouse epiphyseal growth plate chondrocytes.

Identification and characterization of a third human, rat, and mouse collagen prolyl 4-hydroxylase isoenzyme

Identification and characterization of structural domains of human ERp57: association with calreticulin requires several domains

Improved production of human type II procollagen in the yeast Pichia pastoris in shake flasks by a wireless-controlled fed-batch system

Influence of the oxidoreductase ER57 on the folding of an antibody fab fragment

Inhibition of hypoxia-inducible factor (HIF) hydroxylases by citric acid cycle intermediates: possible links between cell metabolism and stabilization of HIF.

Interactions between lysyl oxidases and ADAMTS proteins suggest a novel crosstalk between two extracellular matrix families

Kinetic Analysis of HIF Prolyl Hydroxylases

Lack of P4H-TM in mice results in age-related retinal and renal alterations.

Lack of activity of recombinant HIF prolyl hydroxylases (PHDs) on reported non-HIF substrates

Loss of Cln5 leads to altered Gad1 expression and deficits in interneuron development in mice

Loss of assembly of the main basement membrane collagen, type IV, but not fibril-forming collagens and embryonic death in collagen prolyl 4-hydroxylase I null mice

Lysyl hydroxylase 2 is a specific telopeptide hydroxylase, while all three isoenzymes hydroxylate collagenous sequences

Lysyl oxidase is essential for normal development and function of the respiratory system and for the integrity of elastic and collagen fibers in various tissues

Lysyl oxidase oxidizes cell membrane proteins and enhances the chemotactic response of vascular smooth muscle cells

Many amino acid substitutions in a hypoxia-inducible transcription factor (HIF)-1alpha-like peptide cause only minor changes in its hydroxylation by the HIF prolyl 4-hydroxylases: substitution of 3,4-dehydroproline or azetidine-2-carboxylic acid for

Missense mutations that cause Bruck syndrome affect enzymatic activity, folding, and oligomerization of lysyl hydroxylase 2

Modelling of translation of human protein disulfide isomerase in Escherichia coli-A case study of gene optimisation.

Muscle composition is regulated by a Lox-TGFβ feedback loop

NMR assignment of the N-terminal domain a of the glycoprotein chaperone ERp57

Notch Downregulation and Extramedullary Erythrocytosis in Hypoxia-Inducible Factor Prolyl 4-Hydroxylase 2-Deficient Mice

Null mutation in P4h-tm leads to decreased fear and anxiety and increased social behavior in mice

Oxygen-dependent ATF-4 stability is mediated by the PHD3 oxygen sensor

P4HA1 mutations cause a unique congenital disorder of connective tissue involving tendon, bone, muscle and the eye.

PHD1 regulates p53-mediated colorectal cancer chemoresistance.

Premature aggregation of type IV collagen and early lethality in lysyl hydroxylase 3 null mice

Proline hydroxylation in collagen supports integrin binding by two distinct mechanisms.

Prolyl 4-hydroxlase activity is essential for development and cuticle formation in the human infective parasitic nematode Brugia malayi

Prolyl 4-hydroxylases, key enzymes in the synthesis of collagens and regulation of the response to hypoxia, and their roles as treatment targets

Prolyl 4-hydroxylases, the key enzymes of collagen biosynthesis ⬇️

Prolyl 4-hydroxylation regulates Argonaute 2 stability

Prolyl and lysyl hydroxylases in collagen synthesis

Prolyl hydroxylase domain 2 reduction enhances skeletal muscle tissue regeneration after soft tissue trauma in mice

Recombinant collagen trimers from insect cells and yeast

Relevance of posttranslational modifications for the arthritogenicity of type II collagen

Role of prolyl hydroxylation in the molecular interactions of collagens