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Tiêu đề Oxygen-dependent regulation of hypoxia-inducible factors by prolyl and asparaginyl hydroxylation
Tác giả David Lando, Jeffrey J. Gorman, Murray L. Whitelaw, Daniel J. Peet
Trường học University of Adelaide
Chuyên ngành Biochemistry
Thể loại Review article
Năm xuất bản 2003
Thành phố Adelaide
Định dạng
Số trang 10
Dung lượng 248,94 KB

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Keywords: oxygen sensing; hypoxia; hydroxylation; transcriptional regulation; hypoxia-inducible factor HIF.. Fax: + 61 8 8303 4348, E-mail: daniel.peet@adelaide.edu.au Abbreviations: ARN

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R E V I E W A R T I C L E

Oxygen-dependent regulation of hypoxia-inducible factors by prolyl and asparaginyl hydroxylation

David Lando1, Jeffrey J Gorman2,*, Murray L Whitelaw1and Daniel J Peet1

1

Department of Molecular BioSciences (Biochemistry) and the Centre for Molecular Genetics of Development,

University of Adelaide, Australia;2CSIRO Health Sciences and Nutrition, Parkville, Victoria, Australia

To sustain life mammals have an absolute and continual

requirement for oxygen, which is necessary to produce

energy for normal cell survival and growth Hence,

main-tainingoxyg en homeostasis is a critical requirement and

mammals have evolved a wide range of cellular and

phy-siological responses to adapt to changes in oxygen

avail-ability In the past few years it has become evident that the

transcriptional protein complex hypoxia-inducible factor

(HIF) is a key regulator of these processes In this review we

will focus on the way oxygen availability regulates HIF proteins and in particular we will discuss the way oxygen-dependent hydroxylation of specific amino acid residues has been demonstrated to regulate HIF function at the level of both protein stability and transcriptional potency.

Keywords: oxygen sensing; hypoxia; hydroxylation; transcriptional regulation; hypoxia-inducible factor (HIF).

Introduction

The development of complex cardiovascular, respiratory

and hemopoietic systems in mammals provides a means to

efficiently capture and deliver oxygen (O2) from the

environment to every cell of the body While a sufficient

supply of oxygen is essential for energy production, too

much oxygen in the form of free radicals (i.e superoxide,

OH–) can be detrimental [1] Therefore to maximize oxygen

use, as well as at the same time minimize the impact of

oxygen free radicals, cells have developed mechanisms to

maintain oxygen concentrations within a narrow

physiological range To achieve this mammals regulate

oxygen consumption and levels by a combination of both

cellular and systemic processes For example, when oxygen is limiting(hypoxia) individual cells decrease oxidative phos-phorylation and rely on glycolysis as the primary means of ATP production To facilitate this switch to glycolysis cells up-regulate the expression of a select set of genes, such as those encoding glycolytic enzymes and glucose transporters [2] Other hypoxic responses monitor global oxygen levels and effect system wide changes in tissue oxygen availability For instance, the hypoxic induction of the hormone erythropoietin (Epo) by the kidney stimulates red blood cell production to increase the oxygen carrying capacity of the blood [2] Tissues and cells experiencingreduced oxyg en supply, like those associated with wound healing, increase the levels of the angiogenic cytokine vascular endothelial growth factor (VEGF) VEGF then acts on endothelial cells

to stimulate the proliferation of new blood vessels, which in turn help maintain an adequate supply of oxygen [3] However, in many disease states such as cancer, stroke and heart attack these same oxygen delivery systems can become misregulated and hypoxia becomes a major component of the pathophysiology of these diseases [4].

For many years the Epo system was used to study the molecular mechanisms associated with the induction of hypoxia responsive genes and from these investigations the hypoxia-inducible factor (HIF) was identified as a key transcriptional hypoxic regulator of Epo [5,6] Subsequent research has now found that a large number of other hypoxia-inducible genes (Fig 1) are also induced by HIF under hypoxic conditions, revealingthat HIF functions as a master transcriptional regulator of the adaptive response to hypoxia [7–53].

Hypoxia-inducible factor

The HIF transcriptional complex is a heterodimer consist-ingof one of three alpha subunits (HIF-1a, HIF-2a or HIF-3a) and a beta subunit called ARNT [6,54–57].

Correspondence toD J Peet, Department of Molecular

BioSciences (Biochemistry) and the Centre for Molecular Genetics

of Development, University of Adelaide, Adelaide, South Australia,

5005 Australia Fax: + 61 8 8303 4348,

E-mail: daniel.peet@adelaide.edu.au

Abbreviations: ARNT, aryl hydrocarbon nuclear translocator;

bHLH, basic helix-loop-helix; CAD, carboxy-terminal transactivation

domain; CBP, CREB bindingprotein; CH, cysteine-histidine;

CO, carbon monoxide; CREB, cyclic AMP-response element binding

protein; DMOG, dimethyloxalylglycine; Dsfx, desferrioxamine;

Epo, erythropoietin; FIH-1, factor inhibitingHIF-1; HIF,

hypoxia-inducible factor; HPH, HIF prolyl-4-hydroxylase; MAPK, mitogen

activated protein kinase; NAD, amino-terminal transactivation

domain; NO, nitric oxide; ODD, oxygen-dependent degradation

domain; PAS, Per-ARNT-Sim; PHD, prolyl hydroxylase

domain-containingprotein; RLL, arginine-dileucine; VEGF, vascular

endothelial growth factor; VHL, von Hippel-Lindau

*Present address: Institute for Molecular Bioscience, University of

Queensland, St Lucia, Queensland, 4067, Australia

(Received 15 October 2002, revised 13 December 2002,

accepted 3 January 2003)

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Both the alpha and ARNT subunits belongto the basic

helix-loop-helix (bHLH)/Per-ARNT-Sim (PAS) family of

transcription factors The bHLH domain contains the basic

DNA bindingregion and HLH primary dimerization

inter-face The adjacent PAS domain is comprised of

approxi-mately 300 amino acids and is subdivided into two semi

conserved repeat regions designated PAS A and PAS B The

role of the PAS domain is to mediate protein–protein

interactions and act as a second dimerization interface in

conjunction with the HLH motif [58] ARNT is a general

partner protein and is known to heterodimerize with a

number of other bHLH-PAS proteins to form

transcrip-tionally active complexes [59] Biochemical comparison of

the HIF-1a and HIF-2a subunits have revealed that these

proteins share very similar biochemical properties (i.e.

dimerize with ARNT to recognize the same DNA

recogni-tion sequence) but, surprisingly, each subunit controls quite

distinct biological functions during embryo development (i.e.

HIF-1a for vascularization, HIF-2a for catecholamine

production; for a comprehensive review see [60,61]).

Regulation of HIF proteins by hypoxia

One of the major challenges facing the HIF research field

has been to understand the molecular mechanism by which

cells are able to sense oxygen levels and transduce the

physiological signal of reduced oxygen levels to HIF It has

been reported that oxygen levels can affect the protein

stability, subcellular localization, DNA bindingcapacity

and transcriptional potency of the HIFa subunits, whereas the ARNT subunit is constitutively expressed and its activity not affected by hypoxia (reviewed in [60,61]) While the HIFa subunits may be subject to numerous levels of regulation by oxygen, it has been the recent analysis of HIF-1a and HIF-2a protein stability and transactivation potency that have provided the greatest insights into oxygen sensing and regulation.

HIF protein stability Initial biochemical analysis of HIF-1a revealed that this protein was subject to rapid turnover and degradation at normoxia, whereas hypoxia blocked degradation leading to the accumulation of the HIF-1a protein [62,63] Treatment with proteasomal inhibitors and mutation of the ubiquitin activatingenzyme E1 revealed that HIF-1a was being degraded by the ubiquitin proteasome pathway under normoxic conditions [64] Subsequent studies mapped the instability region of HIF-1a to a domain of approximately

200 amino acids located carboxy-terminal to the PAS domain [65] This region was subsequently called the oxygen-dependent degradation domain (ODD) and removal of the entire ODD rendered HIF-1a stable at normoxia Likewise, analysis of HIF-2a revealed that it was also subject to proteasomal degradation at normoxia [66] via a similar ODD like region [67].

A hallmark of von Hippel-Lindau (VHL) disease is the high degree of vascularization, which is due to the

Fig 1 Hypoxia-inducible factor (HIF) target genes and their roles in oxygen homeostasis Hypoxia activates the HIF complex which binds to hypoxia response elements (HREs) containingthe core recognition sequence 5¢-RCGTG found in numerous genes involved

in a variety of cellular and system wide responses to low oxygen stress

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constitutive expression of a large number of hypoxia

inducible genes such as VEGF (reviewed in [68]) Because

VEGF and other hypoxia inducible genes are known HIF

targets, these observations raised the question of whether

VHL disease and HIF were somehow related Usingvarious

cell lines deficient in VHL, Maxwell and coworkers

demon-strated that VHL–/– cells expressed increased levels of

endogenous HIF-1a and HIF-2a protein [69] Moreover,

the protein levels of HIF-1a and HIF-2a could not be further

induced by hypoxia in VHL–/–cells Reintroduction of VHL

back into these VHL deficient cell lines resulted in a reduction

of normoxic endogenous HIF-1a and HIF-2a protein that

could now be induced by hypoxia [69] Further analysis

demonstrated that VHL could physically interact with the

HIFa subunits via the ODD [69], and the VHL complex

functioned as an ubiquitin ligase capable of ubiquitylating the

HIFa subunits at normoxia and targeting them for

destruc-tion by the proteasome [70–73] Alongwith hypoxia, iron

chelatingagents such as desferrioxamine (Dsfx) were also

able to block VHL interaction, suggesting a requirement

for iron in the normoxic degradation of HIFa subunits [69].

HIF transactivation

Deletion analysis of HIF-1a protein revealed that HIF-1a

contained two transactivation regions, termed the

amino-terminal transactivation domain (NAD) and the

carboxy-terminal transactivation domain (CAD) [74,75] Functional

analysis revealed that the activity of both the NAD and

CAD were enhanced by hypoxia treatment Since the NAD

overlaps with the ODD its increase in transcriptional

activity at hypoxia was largely attributed to increased

protein stability [75] However, the increase in

transcrip-tional activity of the CAD was not attributed to changes in

protein level [75] Instead, hypoxia was suggested to

promote the recruitment of transcriptional coactivator

proteins such as CBP/p300 [76–78], steroid receptor

coac-tivator-1 (SRC-1), and transcription intermediary factor 2

(TIF2) [77] to the CAD region Likewise, analysis of

HIF-2a revealed that it also contained two transactivation

domains with similar organization [67,79] and, as with

HIF-1a, the CAD of HIF-2a was also inducible by hypoxia.

While the transactivation capability of HIF-3a is poorly

characterized, sequence alignment suggests that HIF-3a

lacks an analogous inducible CAD region [57].

Therefore, the transactivation potency of HIF-1a and

HIF-2a CADs is negatively regulated by oxygen

independ-ent of protein stability, revealingthat alongwith the ODD

there exists a second oxygen sensing region near the

carboxy-terminus Moreover, like the ODD the CAD is also sensitive

to iron antagonists (i.e cobalt chloride, Dsfx) suggesting that

the mechanism of regulation of both domains involves a

common iron dependent process [74,75,79].

Regulation of HIFa subunits

by oxygen-dependent prolyl and asparaginyl

hydroxylation

A variety of oxygen sensors have been described for

prokaryotes and yeast [80]; however, for many years the

nature of the cellular oxygen sensor in higher organisms

remained elusive A number of interestingmodels have been

proposed to explain how mammalian cells could sense oxygen, including those that involve the hemoprotein, NADPH oxidoreductase, members of the mitochondrial electron transport chain [81], or oxygen-regulated potassium channels [82] Disappointingly, however, when investigated

in more detail none of these models could clearly demon-strate how HIF activation was beinguniversally regulated.

To better understand the mechanism of oxygen sensing and signal transduction, considerable effort over the last few years has focused on decipheringthe biochemical param-eters by which the ODD and CAD were beingregulated by low oxygen levels.

By employingboth protein interaction and ubiquitylation assays the major VHL bindingregion was narrowed down

to a 20 amino acid stretch within the ODD of both HIF-1a and HIF-2a [70,71,83] Treatment with hypoxia was able to induce the dissociation of VHL from HIF-1a, sug g esting that some cellular activity in normoxic cells maybe respon-sible for promotingVHL association [83] In support of this hypothesis, a synthetic peptide comprisingof the minimal VHL bindingmotif of HIF-1a was unable to interact with VHL unless pretreated with normoxic cell extracts [84–86] Likewise, similar biochemical experiments with the CAD demonstrated that a cellular activity was targeting the CAD for repression at normoxia, and hypoxia blocked this cellular activity, thereby promotingthe recruitment of coactivator proteins such as CBP/p300 [78].

Then, in an elegant set of experiments, a number of groups concurrently demonstrated that the cellular activity responsible for targeting HIFa for degradation was the enzymatic hydroxylation of a specific proline residue within the ODD Hydroxylation of this proline residue was shown

to promote high affinity binding of VHL protein [84–86] Subsequently, a second proline hydroxylation site was identified within the ODD and was also shown to promote VHL bindingin a hydroxylation dependent manner [87] Surprisingly, in a similar but distinct mechanism, an enzymatic hydroxylase activity was also found to specifically modify the CAD at normoxia to block p300 binding[88,89] However, in contrast to the ODD, the hydroxylation activity targeting the CAD was found to occur on an asparagine residue [88] While the CAD contains a number

of proline residues no evidence of hydroxylation of these proline sites has ever been found (D Lando, J J Gorman,

M L Whitelaw & D J Peet, unpublished observations) Hydroxyproline and hydroxyasparagine therefore control HIFa activity by regulating protein–protein interactions; hydroxyproline provides a dockingsite for VHL binding while hydroxyasparagine prevents binding of the coacti-vator p300 Finally, the longsoug ht after links between oxygen availability and iron in the regulation of HIFa protein stability and transactivation potential were realized when it was demonstrated that hypoxia and iron chelators could block hydroxylation of both the proline and aspara-gine residues, regulating the association of VHL with the ODD [84–87] and p300 with the CAD [88], respectively HIF prolyl and asparaginyl hydroxylases

Prior to the discovery of the HIF hydroxylases, the best characterized prolyl and asparaginyl hydroxylases were those that modify proline residues in collagen [90] and

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asparagine or aspartic acid residues in epidermal growth

factor (EGF)-like domains [91] The structures of the

catalytic domains of some of these iron dependent

hydroxylases have been solved and reveal a conserved

double-stranded b-helix enzymatic core commonly referred

to as a jellyroll Within this enzymatic core is a critical

2-His1-carboxylate motif (His-X-Asp/Glu…His),

respon-sible for coordinatingthe bindingof the iron atom [92] By

usinga combination of protein database mining , and

genetic and biochemical assays, three novel HIF prolyl

hydroxylase enzymes (designated prolyl hydroxylase

do-main-containingproteins (PHDs) 1, 2 and 3 [93], or HIF

prolyl hydroxylases (HPHs) 3, 2 and 1, respectively [94]), and one HIF asparaginyl hydroxylase enzyme called factor-inhibitingHIF-1 (FIH-1) [88,95] were identified and shown to hydroxylate the key proline and asparagine residues in HIFa The enzymatic reactions carried out by the PHD/HPHs and FIH-1 revealed that the hydroxyla-tion reachydroxyla-tion requires oxygen (in the form of dioxygen O2), iron (Fe2+) and the cofactor 2-oxoglutarate The hydroxy-lation reaction is inherently dependent on ambient oxygen because the oxygen atom used to form the proline and asparagine hydroxyl groups is derived directly from molecular oxygen [95,96] The cofactor 2-oxoglutarate is required because it undergoes a decarboxylation reaction, consumingthe remainingoxygen atom to form succinate and CO2(Fig 2).

Therefore, the rapid turnover and transcriptional silen-cingof the HIFa protein subunits involves oxygen-depend-ent prolyl and asparaginyl hydroxylation by the PHD/ HPHs and FIH-1 proteins, respectively These modifica-tions then serve as signals for either VHL binding and polyubiqutylation which targets the HIFa subunits for proteasomal degradation, or blocking coactivator proteins such as p300 from bindingthe CAD (Fig 3) The import-ance of the PHD/HPH-HIFa-VHL pathway in the oxygen response is further confirmed by the findingthat compo-nents of this pathway are functionally conserved in Caenorhabditis elegans [93] and Drosophila [94,97] Cur-rently FIH-1 homologues have been predicted to exist in

C elegans and Drosophila [98] but their functionality awaits further confirmation.

Oxygen sensing

By conductingreactions in a controlled oxygen environment

it has been demonstrated that the activity of the PHD/ HPHs are sensitive to graded oxygen levels [93] Moreover,

Fig 2 General reaction scheme for oxygen-dependent hydroxylation by

PHD/HPH and FIH-1 hydroxylases The hydroxylation of target

substrates requires dioxygen (O2), iron [Fe(II)] and the cofactor

2-oxoglutarate During catalysis the substrate accepts one oxygen

atom while 2-oxoglutarate undergoes a decarboxylation reaction

consumingthe remainingoxygen atom to form succinate and CO2

Fig 3 Regulation of hypoxia inducible factors (HIF) by oxygen-dependent hydroxylation In oxygenated conditions (normoxia) the asparaginyl and HIF prolyl hydroxylases (FIH-1 and PHD/HPH) hydroxylate (OH) HIFa on specific asparagine (Asn) and proline (Pro) residues, blocking transactivation and targeting HIFa for destruction by ubiquitin proteasome pathway, respectively Hypoxia and iron antagonists block both PHD/HPH and FIH-1 activity, then HIFa escapes destruction and recruits coactivators (CBP/p300) to induce hypoxia target genes Oxygen-dependent degradation domain (ODD), carboxy-terminal activation domain (CAD), von Hippel Lindau protein (VHL), cobalt chloride (Co), desferrioxamine (Dfrx), 2¢-2-dipyridyl (DP)

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the decreased hydroxylation seems to mirror the progressive

cellular increase in HIFa protein levels observed when cells

are subjected to similar oxygen gradients [99] Therefore, it

has been speculated that the PHD/HPHs represent primary

oxygen sensors Likewise, it has been suggested, but not yet

demonstrated, that FIH-1 catalytic activity is also sensitive

to oxygen gradients [95] Evidence so far suggests that while

the PHD/HPHs may act more as gross regulators of HIF

activity, FIH-1 may play a more subtle role For example,

when HIFa subunits are fully stabilized, as observed in

VHL-deficient cells [69], then FIH-1 activity is saturated by

the large excess of HIF protein, rendering the CAD active at

normoxia However, it is likely that FIH-1 plays a crucial

role in regulating the transcriptional activity of relatively

smaller, yet physiologically relevant, amounts of stabilized

HIFa, such as that produced in response to growth factors.

Interestingly, reporter assays have demonstrated that

overexpression of FIH-1 is still able to partially inhibit CAD

activity under hypoxic conditions [98,100] As

hydroxyla-tion of the CAD under hypoxic condihydroxyla-tions is essentially

absent [88] this inhibition is unlikely to be mediated by

hydroxylation It may be due to direct competition for p300

bindingto the CAD, the recruitment of other factors such as

histone deacetylases or VHL [98], or may well be an artefact

of overexpression.

Structural implications

Synthetic peptides composed of the minimal VHL binding

motif of HIF-1a chemically synthesized with a

4-hydroxy-proline at the critical 4-hydroxy-proline position were shown to bind

VHL protein [84–86] Subsequent structural analysis

dem-onstrated that the tight binding of VHL to the hydroxylated

peptide was due to the 4-hydroxyproline residue forming

critical hydrogen bonds with residues in VHL [101,102].

Taken together, these observations suggest that VHL can

specifically recognize a 4-hydroxyproline.

Recently the solution structures of the CH1 domains of

p300 or CBP bound to the CAD of HIF-1a were solved

[103,104] Analysis of the bound complex revealed that the

CAD remains relatively extended, wrappingitself around

the globular structure of the CH1 domain in a hand grasp or

vice like manner The critical asparagine residue (Asn803) in

the CAD of HIF-1a is found buried deep within the

molecular interface and nearly 45% of its surface is

concealed in the interface Asparagine 803 forms two side

chain hydrogen bonds with aspartic acid residues in the

CAD (Asp799) and the CH1 domain that help to stabilize

the complex Analysis of the asparagine and aspartic acid

hydroxylation products in the EGF like domains of other

hydroxylated proteins has revealed that the hydroxyl group

is attached to the b carbon in the erythro isoform [105] If

the asparagine in the CAD is also hydroxylated on the

b-carbon, either erythro or threo isoforms are predicted to

destabilize the p300/CBP- HIFa complex formation

[103,104] Apart from hydroxylatingthe asparagine on the

b carbon, FIH-1 could also potentially hydroxylate the

asparagine on the side chain amide nitrogen to form a

hydroxyamic acid However, it has been recently reported

that the asparagine 803 in HIF-1a is indeed hydroxylated on

the b-carbon Surprisingly, this hydroxylation is in the threo

isoform [106], unlike the previously characterized EGF-like

domain asparaginyl hydroxylases, which hydroxylate exclusively in the erythro position [105] Also, unlike other asparaginyl hydroxylase enzymes, which can hydroxylate both asparagine and aspartic acid residues, the FIH-1 enzyme was shown to have a clear preference for asparagine

in the CAD of HIF-1a [95] If the asparagine in the CAD was substituted with an aspartic acid residue, FIH-1 hydroxylase activity for the aspartic acid residue was only 7% of that obtained with asparagine This clear difference

in amino acid specificity and the production of threo rather than erythro isomers suggests that FIH-1 belongs to a new subfamily of 2-oxoglutarate-dependent asparaginyl hydroxylases.

Substrate specificity The three PHD/HPH enzymes have been shown to hydroxylate specific proline residues within the context of two strongly conserved LXXLAP* motifs (P* indicates hydroxy proline acceptor) within the ODD [87,93] While in-vitro substrate analysis has revealed that the three PHD/ HPHs have differinghydroxylatingactivity towards the proline residue, they also unfortunately report conflicting evidence showingdifferent enzymes as havinghig hest activity (i.e PHD-2/HPH-2 [107] vs PHD-3/HPH-1 [94]) Nevertheless, it will now be interestingto determine which PHD/HPH enzymes are the main regulator of HIFa hydroxylation in the cell under physiological conditions Expression analysis of the three PHD/HPHs in HeLa cells has revealed that all three mRNAs are expressed at normoxia with PHD-1/HPH-3 exhibitingthe g reatest expression [93] Interestingly, the expression of PHD-2/ HPH-2 and PHD-3/HPH-1, but not PHD-1/HPH-3 are induced by hypoxia [93], suggesting a possible role for these inducible enzymes in a negative feedback pathway respon-sible for enhanced degradation of HIFa after re-oxygen-ation.

Intriguingly, interaction assays have shown that FIH-1 interacts with the CAD of HIF-1a in a region that does not contain the hydroxylated asparagine residue [98] This asparagine residue is actually located approximately 20–30 residues carboxy-terminal to the putative FIH-1 binding region This suggests that for FIH-1 to efficiently hydroxy-late the asparagine residue in HIF-1a it may need to bind to

a reg ion in HIF-1a adjacent to the asparagine motif To support this notion it has been demonstrated that bindingof p300 is not enhanced by the hydroxylase inhibitor dime-thyloxalylglycine (DMOG), or iron antagonists, when the putative FIH-1 bindingregion is removed [89] A region spanningthe FIH-1 bindingsite in HIF-1a contains an arginine-dileucine (RLL) motif that has previously been shown to be critical for the normal silencingof the HIF-1a CAD at normoxia [79] An analogous RLL motif-contain-ingreg ion also operates in a similar silencingfashion in HIF-2a [79], suggesting that this region of both HIF-1a and HIF-2a may contain important elements for targeting FIH-1 The findingthat FIH-1 must bind to HIF-1a in a region away from the critical asparagine residue for efficient hydroxylation may help explain the longknown phenom-enon that uncouplingthe HIF-1a CAD containingresidues 786–826 from the adjacent inhibitory domain results in a highly active CAD under normoxic conditions [74,75] that

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binds strongly to CBP/p300 irrespective of hypoxia

treat-ment [78].

As well as interactingwith the CAD region FIH-1 has

also been shown to interact with VHL via its b domain [98].

Initially this interaction was thought to be important for the

repressive activity of FIH-1 on CAD function [98]; however,

a more recent analysis in VHL null cells has shown that

VHL is not critical for FIH-1 repressive activity [89] It is

possible that FIH-1 and VHL complexes may operate in

additional oxygen regulated processes that affect the

transcriptional response of other pathways Interestingly,

both FIH-1 and VHL have been shown to interact with

chromatin modifyinghistone deacetylase (HDAC)

enzymes, which are known to play an important role in

gene repression [98] Together these observations raise the

possibility that FIH-1 may have multiple roles other than

just regulating the CAD of HIF-1a and HIF-2a.

Other mechanisms of activation

The induction of HIF activity by well established agents

such as hypoxia, cobaltous ions and iron chelators can be

easily explained by the findingthat the PHD/HPHs and

FIH-1 are members of the 2-oxoglutarate-dependent family

of hydroxylase enzymes that utilize iron and oxygen to

modify their target amino acid residues However, it has

also been reported that HIF activity is influenced by

particular gas molecules (i.e NO, CO), reactive oxygen

species (i.e H2O2), and phosphorylation events (i.e p38

MAPK), although the understanding of how these

proces-ses may influence PHD/HPHs and FIH-1 function and HIF

activity is less clear (reviewed in [7]) NO is a known

analogue of dioxygen and analysis of the non heme iron

(Fe2+) dependent isopenicillin N synthase enzyme, a closely

related oxygenase to the 2-oxoglutarate family, has

dem-onstrated that NO can bind to the iron centre of this enzyme

[108] Because NO has only one available oxygen atom for

use in catalysis and 2-oxoglutarate-dependent dioxygenases

normally require two oxygen atoms for completing the

hydroxylation of their substrates (Fig 2), the binding of NO

to the catalytic core of PHD/HPHs and FIH-1 may block

enzymatic activity, explainingthe reported positive effects of

NO on HIF activity [109] The hydroxylation of substrates

by the collagen prolyl-4-hydroxylase has been shown to be

inhibited by the artificial generation of radicals at the

enzyme active site [110] Thus, the effects of reactive oxygen

species on HIF stability [64] and transactivation [62] may

relate to the alteringof the redox balance of the cell, which

then may affect the catalytic activity of PHD/HPHs and

FIH-1 Finally, it is possible that these other reported agents

that regulate HIF activity may target components of the

VHL ubiquitin ligase or CBP/p300 coactivator complex, or

even the PHD/HPHs and FIH-1 enzymes directly.

Therapeutic benefits

Hypoxia constitutes a major component of many disease

states and can have both a proliferative (cancer) or

damaging affect (stroke, heart attack) on disease

pathogene-sis [4] Therefore, it has been suggested that inducing HIF

activity may be beneficial for stroke and heart attack victims

as this would help promote vascularization of damaged tissue Conversely, blockingHIF activity may be advant-ageous in inhibiting cancer progression as this would help starve growing tumours of oxygen and nutrient supply Coupled with previous studies that have provided Ôproof of principleÕ that targeting HIF stability [111] and transactiva-tion [78] can enhance oxygen delivery and inhibit cancer progression, respectively, it is reasoned that HIF is an attractive target for pharmaceutical manipulation With the discoveries that HIF stability and transcriptional activity are controlled by two distinct modifications (prolyl and aspar-aginyl hydroxylation) the development of small molecule drugs to selectively target HIF to differentially modulate its activity should be possible For instance, while it has been demonstrated that the biological activity of FIH-1 requires 2-oxoglutarate [95,100], an unusual feature of FIH-1 is that

it lacks an arginine or lysine residue located on the eighth

b strand of the enzymatic core These conserved residues have previously been demonstrated to be involved in binding5-carboxylate of 2-oxog lutarate in many other 2-oxoglutarate dependent enzymes [92] Because these 2-oxoglutarate binding residues are conserved in the PHD/HPHs [93,94], it provides further evidence that

FIH-1 represents a new structural submember of the 2-oxoglu-tarate dependent enzyme family, and raises the possibility that selective agonists and antagonists for PHD/HPHs and FIH-1 can be developed Furthermore, a preliminary study has found that certain well-established inhibitors of the collagen prolyl hydroxylase enzymes do not inhibit PHD/ HPH activity, suggesting that it may be possible to design pharmacological inhibitors that can selectively target the HIF prolyl hydroxylases [112].

Global oxygen sensing by protein hydroxylation? Apart from the HIF pathway and HIFa subunits, the regulation and activity of a large number of other cellular processes and proteins have also been demonstrated to be influenced by oxygen availability For example, chronic hypoxia is known to extend the replicative life span of certain cell types such as vascular smooth muscle cells [113] A recent study attributed this increase in long-term proliferation to enhanced hypoxic phosphorylation of the telomerase cata-lytic component TERT [114] Apart from the putative hypoxia regulated kinase that phosphorylates TERT, the activity of a number of other protein kinases have also been shown to be regulated by hypoxia These include p44/ p42MAPK [115], p38 MAPK [116,117] and diacylglycerol kinase [118] Likewise, the stability of certain messenger RNAs, such as VEGF [119,120], are also known to be increased under hypoxic stress, while the splicingof specific alternative mRNA transcripts has recently been shown to be influenced by low oxygen tension [121] While the mechanism

by which low oxygen stress controls these other processes is unknown it will now be of great interest to determine whether the PHD/HPHs or FIH-1 are involved, or if additional oxygen sensing proteins exist that utilize hydroxylation to modify their target substrates The use of pharmacological inhibitors such as DMOG should now allow quick and easy analysis of the contribution of post-translational hydroxy-lation in other oxygen sensitive processes.

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To date three prolyl and one asparaginyl hydroxylase

enzymes have been discovered that can target different

domains of the HIFa subunits, affectingdistinct steps in the

induction of the HIF complex The numerous enzymes and

their various targets may have evolved to help manipulate

the magnitude of the HIF transcriptional response by

providinga variable mechanism to g radually alter the

activity of the HIFa subunits in response to subtle changes

in oxygen levels Furthermore, other studies have suggested

that the nuclear accumulation of HIFa subunits may also be

oxygen regulated [76], and it will now be interesting to

establish if this or other components of HIF regulation are

also influenced by the above or other hydroxylation

mediated events.

Acknowledgements

D J P is the W Bruce Hall Cancer Research Fellow supported by the

Cancer Council of South Australia, and this work was also supported

by the National Heart Foundation and National Health and Medical

Research Council of Australia

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