Pharmacological chaperones for misfolded gonadotropin-releasing hormone receptors.

PubWeight™: 0.91‹?›

🔗 View Article (PMC 3235646)

Published in Adv Pharmacol on January 01, 2011

Authors

P Michael Conn1, Alfredo Ulloa-Aguirre

Author Affiliations

1: Oregon National Primate Research Center, Oregon Health & Science University, Beaverton, Oregon, USA.

Articles citing this

The role of autophagy in neurodegenerative disease. Nat Med (2013) 4.46

Quality control autophagy degrades soluble ERAD-resistant conformers of the misfolded membrane protein GnRHR. Mol Cell (2014) 1.27

Chemical chaperone TUDCA preserves cone photoreceptors in a mouse model of Leber congenital amaurosis. Invest Ophthalmol Vis Sci (2012) 0.97

Pharmacological chaperoning: a primer on mechanism and pharmacology. Pharmacol Res (2014) 0.92

Current and future applications of GnRH, kisspeptin and neurokinin B analogues. Nat Rev Endocrinol (2013) 0.91

Ubiquitination of G protein-coupled receptors: functional implications and drug discovery. Mol Pharmacol (2012) 0.80

Minireview: ubiquitination-regulated G protein-coupled receptor signaling and trafficking. Mol Endocrinol (2013) 0.80

Using pharmacological chaperones to restore proteostasis. Pharmacol Res (2014) 0.79

A small molecule agonist THIQ as a novel pharmacoperone for intracellularly retained melanocortin-4 receptor mutants. Int J Biol Sci (2014) 0.79

Ipsen 5i is a Novel Potent Pharmacoperone for Intracellularly Retained Melanocortin-4 Receptor Mutants. Front Endocrinol (Lausanne) (2014) 0.77

Rescue of misrouted GnRHR mutants reveals its constitutive activity. Mol Endocrinol (2012) 0.77

Mutations in G protein-coupled receptors that impact receptor trafficking and reproductive function. Mol Cell Endocrinol (2013) 0.77

Inside job: ligand-receptor pharmacology beneath the plasma membrane. Acta Pharmacol Sin (2013) 0.76

Regulation of GPCR expression through an interaction with CCT7, a subunit of the CCT/TRiC complex. Mol Biol Cell (2016) 0.75

Pharmacoperone rescue of vasopressin 2 receptor mutants reveals unexpected constitutive activity and coupling bias. PLoS One (2017) 0.75

Articles cited by this

(truncated to the top 100)

Molecular chaperones in the cytosol: from nascent chain to folded protein. Science (2002) 15.09

Quality control in the endoplasmic reticulum. Nat Rev Mol Cell Biol (2003) 9.85

The structure and function of G-protein-coupled receptors. Nature (2009) 8.08

Allosteric modulators of GPCRs: a novel approach for the treatment of CNS disorders. Nat Rev Drug Discov (2009) 6.69

Heterodimerization is required for the formation of a functional GABA(B) receptor. Nature (1998) 5.59

Converging concepts of protein folding in vitro and in vivo. Nat Struct Mol Biol (2009) 5.47

Detection of beta 2-adrenergic receptor dimerization in living cells using bioluminescence resonance energy transfer (BRET). Proc Natl Acad Sci U S A (2000) 4.46

ER chaperones in mammalian development and human diseases. FEBS Lett (2007) 4.40

GABA(B)-receptor subtypes assemble into functional heteromeric complexes. Nature (1998) 4.33

Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet (2007) 4.13

A trafficking checkpoint controls GABA(B) receptor heterodimerization. Neuron (2000) 3.98

A peptide derived from a beta2-adrenergic receptor transmembrane domain inhibits both receptor dimerization and activation. J Biol Chem (1996) 3.77

Quality control in the endoplasmic reticulum protein factory. Nature (2003) 3.58

Dimerization: an emerging concept for G protein-coupled receptor ontogeny and function. Annu Rev Pharmacol Toxicol (2002) 3.45

Quantitative assessment of beta 1- and beta 2-adrenergic receptor homo- and heterodimerization by bioluminescence resonance energy transfer. J Biol Chem (2002) 3.40

Identification of ligands for olfactory receptors by functional expression of a receptor library. Cell (1998) 3.33

Oligomerization of G-protein-coupled transmitter receptors. Nat Rev Neurosci (2001) 3.30

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures. Biochemistry (1981) 3.08

Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy. Trends Mol Med (2005) 2.96

Mutation in the follicle-stimulating hormone receptor gene causes hereditary hypergonadotropic ovarian failure. Cell (1995) 2.89

Proteasome-dependent endoplasmic reticulum-associated protein degradation: an unconventional route to a familiar fate. Proc Natl Acad Sci U S A (1996) 2.82

The fourth transmembrane segment forms the interface of the dopamine D2 receptor homodimer. J Biol Chem (2002) 2.75

Regulation of protein secretion through controlled aggregation in the endoplasmic reticulum. Science (2000) 2.70

Quality control and protein folding in the secretory pathway. Annu Rev Cell Dev Biol (2003) 2.69

A family with hypogonadotropic hypogonadism and mutations in the gonadotropin-releasing hormone receptor. N Engl J Med (1997) 2.66

The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation. J Cell Sci (2002) 2.65

Emerging role of homo- and heterodimerization in G-protein-coupled receptor biosynthesis and maturation. Trends Pharmacol Sci (2005) 2.63

Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78. Proc Natl Acad Sci U S A (2010) 2.40

Nephrogenic diabetes insipidus. Annu Rev Physiol (2001) 2.38

Identification of G protein-coupled receptor genes from the human genome sequence. FEBS Lett (2002) 2.33

A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system. J Biol Chem (2002) 2.26

Episodic luteinizing hormone secretion in man. Pulse analysis, clinical interpretation, physiologic mechanisms. J Clin Invest (1973) 2.23

Gonadotropin-releasing hormone receptors. Endocr Rev (2004) 2.22

Pharmacologic chaperones as a potential treatment for X-linked nephrogenic diabetes insipidus. J Am Soc Nephrol (2005) 2.14

The melanocortin-4 receptor: physiology, pharmacology, and pathophysiology. Endocr Rev (2010) 2.09

Pharmacologic rescue of conformationally-defective proteins: implications for the treatment of human disease. Traffic (2004) 2.05

Homodimerization of the beta2-adrenergic receptor as a prerequisite for cell surface targeting. J Biol Chem (2004) 2.03

Mechanism of transdominant inhibition of CCR5-mediated HIV-1 infection by ccr5delta32. J Biol Chem (1997) 1.99

Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds. J Biol Chem (2001) 1.99

Regulation of G protein-coupled receptor export trafficking. Biochim Biophys Acta (2006) 1.99

G protein-coupled receptor trafficking in health and disease: lessons learned to prepare for therapeutic mutant rescue in vivo. Pharmacol Rev (2007) 1.98

Role of CCR5 in infection of primary macrophages and lymphocytes by macrophage-tropic strains of human immunodeficiency virus: resistance to patient-derived and prototype isolates resulting from the delta ccr5 mutation. J Virol (1997) 1.98

Pharmacological chaperones: potential treatment for conformational diseases. Trends Endocrinol Metab (2004) 1.96

Specificity and sensitivity of a human olfactory receptor functionally expressed in human embryonic kidney 293 cells and Xenopus Laevis oocytes. J Neurosci (1999) 1.96

Membrane trafficking of G protein-coupled receptors. Annu Rev Pharmacol Toxicol (2004) 1.94

Export from the endoplasmic reticulum represents the limiting step in the maturation and cell surface expression of the human delta opioid receptor. J Biol Chem (2000) 1.91

Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. Am J Physiol Cell Physiol (2006) 1.82

G protein-coupled receptor dimerisation: molecular basis and relevance to function. Biochim Biophys Acta (2006) 1.76

Cell surface expression of alpha1D-adrenergic receptors is controlled by heterodimerization with alpha1B-adrenergic receptors. J Biol Chem (2004) 1.75

Constitutive activity and inverse agonists of G protein-coupled receptors: a current perspective. Mol Pharmacol (2003) 1.72

Newly synthesized human delta opioid receptors retained in the endoplasmic reticulum are retrotranslocated to the cytosol, deglycosylated, ubiquitinated, and degraded by the proteasome. J Biol Chem (2000) 1.69

Protein folding and misfolding: mechanism and principles. Q Rev Biophys (2008) 1.68

Monitoring of ligand-independent dimerization and ligand-induced conformational changes of melatonin receptors in living cells by bioluminescence resonance energy transfer. J Biol Chem (2002) 1.68

The role of molecular chaperones in human misfolding diseases. FEBS Lett (2009) 1.68

Prevalence, phenotypic spectrum, and modes of inheritance of gonadotropin-releasing hormone receptor mutations in idiopathic hypogonadotropic hypogonadism. J Clin Endocrinol Metab (2001) 1.65

Correcting temperature-sensitive protein folding defects. J Clin Invest (1997) 1.61

Pharmacological chaperones: a new twist on receptor folding. Trends Pharmacol Sci (2000) 1.61

Gonadotropin-releasing hormone and its analogs. Annu Rev Med (1994) 1.61

Molecular biology of hereditary diabetes insipidus. J Am Soc Nephrol (2005) 1.57

Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H. J Biol Chem (2004) 1.55

Endoplasmic reticulum retention, degradation, and aggregation of olfactory G-protein coupled receptors. Traffic (2003) 1.54

Receptor function, dominant negative activity and phenotype correlations for MC1R variant alleles. Hum Mol Genet (2007) 1.52

Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosa. J Biol Chem (2003) 1.51

Heterodimerization with beta2-adrenergic receptors promotes surface expression and functional activity of alpha1D-adrenergic receptors. J Pharmacol Exp Ther (2004) 1.50

Protein folding in the cell: reshaping the folding funnel. Trends Biochem Sci (2004) 1.47

Rescue of hypogonadotropic hypogonadism-causing and manufactured GnRH receptor mutants by a specific protein-folding template: misrouted proteins as a novel disease etiology and therapeutic target. J Clin Endocrinol Metab (2002) 1.46

Pharmacological chaperone action on G-protein-coupled receptors. Curr Opin Pharmacol (2004) 1.45

Signaling by G-protein-coupled receptor (GPCR): studies on the GnRH receptor. Front Neuroendocrinol (2008) 1.40

Heterodimerization of V1a and V2 vasopressin receptors determines the interaction with beta-arrestin and their trafficking patterns. Proc Natl Acad Sci U S A (2004) 1.38

Membrane assembly of the cannabinoid receptor 1: impact of a long N-terminal tail. Mol Pharmacol (2003) 1.37

Protein misassembly: macromolecular crowding and molecular chaperones. Adv Exp Med Biol (2007) 1.35

Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J Cell Sci (2001) 1.34

Pharmacochaperones post-translationally enhance cell surface expression by increasing conformational stability of wild-type and mutant vasopressin V2 receptors. J Biol Chem (2004) 1.33

Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs. Biochim Biophys Acta (2006) 1.31

Altered cell surface expression of human MC1R variant receptor alleles associated with red hair and skin cancer risk. Hum Mol Genet (2005) 1.30

Intracellular entrapment of wild-type TSH receptor by oligomerization with mutants linked to dominant TSH resistance. Hum Mol Genet (2005) 1.30

Structure-activity relations of successful pharmacologic chaperones for rescue of naturally occurring and manufactured mutants of the gonadotropin-releasing hormone receptor. J Pharmacol Exp Ther (2003) 1.26

Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator. J Biol Chem (2003) 1.23

Monitoring receptor oligomerization using time-resolved fluorescence resonance energy transfer and bioluminescence resonance energy transfer. The human delta -opioid receptor displays constitutive oligomerization at the cell surface, which is not regulated by receptor occupancy. J Biol Chem (2001) 1.22

Truncated V2 vasopressin receptors as negative regulators of wild-type V2 receptor function. Biochemistry (1998) 1.21

Inherited disorders of GnRH and gonadotropin receptors. Mol Cell Endocrinol (2001) 1.17

Human loss-of-function gonadotropin-releasing hormone receptor mutants retain wild-type receptors in the endoplasmic reticulum: molecular basis of the dominant-negative effect. Mol Endocrinol (2004) 1.17

Regulation of G protein-coupled receptor trafficking by inefficient plasma membrane expression: molecular basis of an evolved strategy. J Biol Chem (2006) 1.17

Protein folding as posttranslational regulation: evolution of a mechanism for controlled plasma membrane expression of a G protein-coupled receptor. Mol Endocrinol (2006) 1.14

Inherited ACTH insensitivity illuminates the mechanisms of ACTH action. Trends Endocrinol Metab (2005) 1.13

Trafficking of G-protein-coupled receptors to the plasma membrane: insights for pharmacoperone drugs. Trends Endocrinol Metab (2009) 1.12

Identification and functional characterization of novel calcium-sensing receptor mutations in familial hypocalciuric hypercalcemia and autosomal dominant hypocalcemia. J Clin Endocrinol Metab (2002) 1.11

Functional characterization of melanocortin-4 receptor mutations associated with childhood obesity. Endocrinology (2003) 1.11

Molecular mechanisms of the neural melanocortin receptor dysfunction in severe early onset obesity. Mol Cell Endocrinol (2005) 1.10

Biochemical and biophysical characterization of serotonin 5-HT2C receptor homodimers on the plasma membrane of living cells. Biochemistry (2004) 1.09

Mammalian olfactory receptors: pharmacology, G protein coupling and desensitization. Cell Mol Life Sci (2009) 1.08

Structural determinants for membrane trafficking and G protein selectivity of a mouse olfactory receptor. J Neurochem (2004) 1.08

Receptor-misrouting: an unexpectedly prevalent and rescuable etiology in gonadotropin-releasing hormone receptor-mediated hypogonadotropic hypogonadism. J Clin Endocrinol Metab (2002) 1.08

Refolding of misfolded mutant GPCR: post-translational pharmacoperone action in vitro. Mol Cell Endocrinol (2007) 1.08

On the control of gonadotropin secretion in the rhesus monkey. Recent Prog Horm Res (1974) 1.08

Structure-activity relationships of G protein-coupled receptors. Arch Med Res (2000) 1.08

Rescue of vasopressin V2 receptor mutants by chemical chaperones: specificity and mechanism. Mol Biol Cell (2005) 1.07

Thyrotropin-releasing hormone receptor processing: role of ubiquitination and proteasomal degradation. Mol Endocrinol (2003) 1.07

Beyond the signal sequence: protein routing in health and disease. Endocr Rev (2004) 1.07

Parallel regulation of membrane trafficking and dominant-negative effects by misrouted gonadotropin-releasing hormone receptor mutants. J Biol Chem (2005) 1.07

Articles by these authors

G protein-coupled receptor trafficking in health and disease: lessons learned to prepare for therapeutic mutant rescue in vivo. Pharmacol Rev (2007) 1.98

Protein:creatinine ratio in random urine samples is a reliable marker of increased 24-hour protein excretion in hospitalized women with hypertensive disorders of pregnancy. Clin Chem (2007) 1.60

Application of new homologous in vitro bioassays for human lactogens to assess the actual bioactivity of human prolactin isoforms in hyperprolactinaemic patients. Clin Endocrinol (Oxf) (2006) 1.44

Trafficking of G-protein-coupled receptors to the plasma membrane: insights for pharmacoperone drugs. Trends Endocrinol Metab (2009) 1.12

In vitro coexpression and pharmacological rescue of mutant gonadotropin-releasing hormone receptors causing hypogonadotropic hypogonadism in humans expressing compound heterozygous alleles. J Clin Endocrinol Metab (2005) 1.06

Urinary prolactin as a reliable marker for preeclampsia, its severity, and the occurrence of adverse pregnancy outcomes. J Clin Endocrinol Metab (2008) 1.01

Dominant-negative action of disease-causing gonadotropin-releasing hormone receptor (GnRHR) mutants: a trait that potentially coevolved with decreased plasma membrane expression of GnRHR in humans. J Clin Endocrinol Metab (2003) 0.98

Evolved regulation of gonadotropin-releasing hormone receptor cell surface expression. Endocrine (2003) 0.95

Molecular basis of hypogonadotropic hypogonadism: restoration of mutant (E(90)K) GnRH receptor function by a deletion at a distant site. J Clin Endocrinol Metab (2002) 0.95

Functional significance of the BBXXB motif reversed present in the cytoplasmic domains of the human follicle-stimulating hormone receptor. Mol Cell Endocrinol (2004) 0.93

Modeling and molecular dynamics simulation of the human gonadotropin-releasing hormone receptor in a lipid bilayer. J Phys Chem B (2008) 0.92

The adapter protein APPL1 links FSH receptor to inositol 1,4,5-trisphosphate production and is implicated in intracellular Ca(2+) mobilization. Endocrinology (2011) 0.92

Functional and structural roles of conserved cysteine residues in the carboxyl-terminal domain of the follicle-stimulating hormone receptor in human embryonic kidney 293 cells. Biol Reprod (2008) 0.89

Increased plasma membrane expression of human follicle-stimulating hormone receptor by a small molecule thienopyr(im)idine. Mol Cell Endocrinol (2008) 0.88

Reproductive axis function and gonadotropin microheterogeneity in a male rat model of diet-induced obesity. Gen Comp Endocrinol (2009) 0.86

Preferential β-arrestin signalling at low receptor density revealed by functional characterization of the human FSH receptor A189 V mutation. Mol Cell Endocrinol (2010) 0.86

Dominant negative effects of human follicle-stimulating hormone receptor expression-deficient mutants on wild-type receptor cell surface expression. Rescue of oligomerization-dependent defective receptor expression by using cognate decoys. Mol Cell Endocrinol (2010) 0.85

Structural determinants in the second intracellular loop of the human follicle-stimulating hormone receptor are involved in G(s) protein activation. Mol Cell Endocrinol (2002) 0.83

Changes in circulating concentrations of soluble fms-like tyrosine kinase-1 and placental growth factor measured by automated electrochemiluminescence immunoassays methods are predictors of preeclampsia. J Hypertens (2012) 0.83

Biochemical mechanism of pathogenesis of human gonadotropin-releasing hormone receptor mutants Thr104Ile and Tyr108Cys associated with familial hypogonadotropic hypogonadism. Mol Cell Endocrinol (2011) 0.81

Intact follicle culture: what it can tell us about the roles of FSH glycoforms during follicle development. Reprod Biomed Online (2002) 0.81

Emerging roles for the FSH receptor adapter protein APPL1 and overlap of a putative 14-3-3τ interaction domain with a canonical G-protein interaction site. Mol Cell Endocrinol (2010) 0.81

Pharmacological chaperones correct misfolded GPCRs and rescue function: protein trafficking as a therapeutic target. Subcell Biochem (2012) 0.81

Conformational effects of Lys191 in the human GnRH receptor: mutagenesis and molecular dynamics simulations studies. J Endocrinol (2009) 0.80

Elevated serum bioactive prolactin concentrations in patients with systemic lupus erythematosus are associated with disease activity as disclosed by homologous receptor bioassays. J Rheumatol (2007) 0.80

Normal testicular function without detectable follicle-stimulating hormone. A novel mutation in the follicle-stimulating hormone receptor gene leading to apparent constitutive activity and impaired agonist-induced desensitization and internalization. Mol Cell Endocrinol (2012) 0.79

Poorly controlled type I diabetes mellitus in young men selectively suppresses luteinizing hormone secretory burst mass. J Clin Endocrinol Metab (2002) 0.79

Effects of FSH and 17beta-estradiol on the transactivation of estrogen-regulated promoters and cell proliferation in L cells. J Steroid Biochem Mol Biol (2005) 0.79

Gonadotropin-releasing hormone receptor activates GTPase RhoA and inhibits cell invasion in the breast cancer cell line MDA-MB-231. BMC Cancer (2012) 0.78

Pituitary-testicular axis function, biological to immunological ratio and charge isoform distribution of pituitary LH in male rats with experimental diabetes. Gen Comp Endocrinol (2009) 0.77

Differential effect of DDT, DDE, and DDD on COX-2 expression in the human trophoblast derived HTR-8/SVneo cells. J Biochem Mol Toxicol (2012) 0.77

Identification of new gonadotrophin-releasing hormone partial agonists. J Endocrinol (2006) 0.76

Stimulating autoantibodies against the angiotensin II type 1 receptor are not associated with preeclampsia in Mexican-Mestizo women. J Hypertens (2010) 0.76

Genistein stimulates fatty acid oxidation in a leptin receptor-independent manner through the JAK2-mediated phosphorylation and activation of AMPK in skeletal muscle. Biochim Biophys Acta (2013) 0.76

[Health Law section in the Gaceta Médica de México]. Gac Med Mex (2012) 0.75

No evidence of the human chorionic gonadotropin-beta gene 5 betaV79M polymorphism in Mexican women. Endocrine (2008) 0.75

Pharmacoperones as a New Therapeutic Approach: In Vitro Identification and In vivo Validation of Bioactive Molecules. Curr Drug Targets (2016) 0.75

In vitro biological-to-immunological ratio of serum gonadotropins throughout male puberty in children with insulin-dependent diabetes mellitus. Endocrine (2007) 0.75

Cloning the uteroglobin gene promoter from the relic volcano rabbit (Romerolagus diazi) reveals an ancient estrogen-response element. Mol Reprod Dev (2012) 0.75

Molecular cloning and functional analysis of the FSH receptor gene promoter from the volcano mouse (Neotomodon alstoni alstoni). Endocrine (2009) 0.75

Norethisterone is bioconverted to oestrogenic compounds that activate both the oestrogen receptor alpha and oestrogen receptor beta in vitro. Eur J Pharmacol (2002) 0.75

cDNA sequence, 5'-flanking region, and promoter activity of the Neotomodon alstoni alstoni Clara cell secretory protein gene. Arch Biochem Biophys (2004) 0.75

Regulation of Clara cell secretory protein gene expression by the CCAAT-binding factor NF-Y. Arch Biochem Biophys (2006) 0.75

[Topics on reproductive health at the beginning of the new millennium]. Gac Med Mex (2008) 0.75

Editorial for the MCE special issue on "Novel endocrine mechanisms in the regulation of reproduction". Mol Cell Endocrinol (2013) 0.75