Nomenclature: LH receptor

Family: Glycoprotein hormone receptors

Annotation status:  image of a green circle Annotated and expert reviewed. Please contact us if you can help with updates. 

Contents

Gene and Protein Information
class A G protein-coupled receptor
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human 7 699 2p21 LHCGR luteinizing hormone/choriogonadotropin receptor 13
Mouse 7 700 17 E4 Lhcgr luteinizing hormone/choriogonadotropin receptor 7
Rat 7 700 6q12 Lhcgr luteinizing hormone/choriogonadotropin receptor 12
Previous and Unofficial Names
Luteinizing hormone receptor
Choriogonadotropin receptor
HHG
LHR
LCGR
LGR2
ULG5
LSHR
hypergonadotropic hypogonadism
luteinizing hormone/choriogonadotropin receptor
LH/CG-R
LSH-R
lutropin-choriogonadotropic hormone receptor
Gpcr19-rs1
LH-R
Database Links
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
GPCRDB
GeneCards
GenitoUrinary Development Molecular Anatomy Project
HomoloGene
Human Protein Reference Database
InterPro
KEGG Gene
OMIM
Orphanet Gene
PharmGKB Gene
PhosphoSitePlus
Protein Ontology (PRO)
RefSeq Nucleotide
RefSeq Protein
TreeFam
UniGene Hs.
UniProtKB
Wikipedia
Natural/Endogenous Ligands
hCG {Sp: Human}
LH {Sp: Human} , LH {Sp: Mouse} , LH {Sp: Rat}
Agonists
Key to terms and symbols Click column headers to sort
Ligand Sp. Action Affinity Units Reference
hCG {Sp: Human} Hs Full agonist 9.9 – 11.8 pKd 8,14
pKd 9.9 – 11.8 [8,14]
LH {Sp: Human} Hs Full agonist 9.9 – 10.9 pIC50 8,14
pIC50 9.9 – 10.9 [8,14]
Agonist Comments
Various thienopyrimidine derivatives are known LH receptor agonists binding with pIC50 values of between 7.7 and 5 [17].

There are considerable differences in affinity when comparing various species combinations of receptors and the natural ligands [5].
Antagonist Comments
Deglycosylated hCGs (human chorionic gonadotropins) are known LH receptor antagonists [3,11].
Primary Transduction Mechanisms
Transducer Effector/Response
Gs family Adenylate cyclase stimulation
References:  6,9
Secondary Transduction Mechanisms
Transducer Effector/Response
Gi/Go family Phospholipase C stimulation
Comments:  LH receptor activation also results in activation of the MAPK pathway (erk1/2 phosphorylation)[21].
References:  9
Tissue Distribution
Ovary, fallopian tubes, endometrium, vaginal wall, vaginal connective tissue fibroblasts, macrophages, brain vessels, microglial cells.
Species:  Human
Technique:  Immunohistochemistry.
References:  2
Placental chorionic villous macrophages.
Species:  Human
Technique:  RT-PCR.
References:  22
Prostate.
Species:  Human
Technique:  RT-PCR.
References:  4
Skin.
Species:  Human
Technique:  Radioligand binding.
References:  15
Adrenal cortex.
Species:  Human
Technique:  in situ hybridisation.
References:  16
Ovary: theca cells, large follicles, corpora lutea, surface epithelium.
Oviduct: serosa and subepithelial cells, smooth muscle.
Uterus: stromal cells of the endometrium and uterine serosa, smooth muscle cells.
Species:  Mouse
Technique:  in situ hybridisation.
References:  28
Ovary > uterus > testes.
Species:  Mouse
Technique:  Radioligand binding.
References:  28
Ovary > uterus > testes.
Species:  Rat
Technique:  Radioligand binding.
References:  28
Tissue Distribution Comments
In many tissues mRNA expression, protein expression and even receptor activity has been found. It is however still under debate whether such functional expression has physiological relevance, since the human and mouse knockouts do not show deficiencies related to this extragonadal expression.
Expression Datasets

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Log average relative transcript abundance in mouse tissues measured by qPCR from Regard, J.B., Sato, I.T., and Coughlin, S.R. (2008). Anatomical profiling of G protein-coupled receptor expression. Cell, 135(3): 561-71. [PMID:18984166] [Raw data: website]

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Functional Assays
Measurement of testosterone levels in rat Leydig cells.
Species:  Rat
Tissue:  Leydig cells of the testes.
Response measured:  Stimulation of testosterone production.
References:  19
Measurement of testosterone levels in mouse interstitial tissue of the testes.
Species:  Mouse
Tissue:  Interstitial tissue of the testes.
Response measured:  Stimulation of testosterone production.
References:  24
Measurement of cAMP and testosterone levels in rat interstitial tissue of the testes.
Species:  Rat
Tissue:  Interstitial tissue of the testes.
Response measured:  Stimulation of cAMP and testosterone production.
References:  20
Measurement of cAMP levels in an L cell line transfected with the mouse LH receptor (LHR 11/6).
Species:  Mouse
Tissue:  L cell line, LHR 11/6.
Response measured:  Stimulation of cAMP production.
References:  7
Measurement of IP, IP2, and IP3 levels in an L cell line transfected with the mouse LH receptor (LHR 11/6).
Species:  Mouse
Tissue:  L cell line, LHR 11/6.
Response measured:  Stimulation of IP, IP2, and IP3 formation.
References:  7
Physiological Functions
Steroidogenesis.
Species:  Rat
Tissue:  Testes.
References:  1
Steroidogenesis.
Species:  Rat
Tissue:  Ovary.
References:  26
Ovulation.
Species:  Mouse
Tissue:  Ovary.
References:  25
Physiological Consequences of Altering Gene Expression
LH receptor knockout mice exhibit anovulation and failure of postnatal sex differentiation.
Species:  Mouse
Tissue: 
Technique:  Gene targeting in embryonic stem cells.
References:  10,27
Phenotypes, Alleles and Disease Models Mouse data from MGI

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Allele Composition & genetic background Accession Phenotype Id Phenotype Reference
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0000010 abnormal abdominal fat pad morphology PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0005189 abnormal anogenital distance PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005189 abnormal anogenital distance PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002660 abnormal caput epididymis morphology PMID: 15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002660 abnormal caput epididymis morphology PMID: 12604639 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002662 abnormal cauda epididymis morphology PMID: 12604639 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001378 abnormal ejaculation PMID: 15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004933 abnormal epididymis epithelium morphology PMID: 12604639 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002631 abnormal epididymis morphology PMID: 12604639 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001119 abnormal female reproductive system morphology PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001119 abnormal female reproductive system morphology PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001145 abnormal male reproductive system morphology PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002783 abnormal ovarian secretion PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001126 abnormal ovary morphology PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001158 abnormal prostate gland morphology PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001919 abnormal reproductive system physiology PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002216 abnormal seminiferous tubule morphology PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002566 abnormal sexual interaction PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0004543 abnormal sperm physiology PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002782 abnormal testicular secretion PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001120 abnormal uterus morphology PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001120 abnormal uterus morphology PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0003531 abnormal vagina development PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001142 abnormal vagina opening PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002770 absent bulbourethral gland PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001134 absent corpus luteum PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001134 absent corpus luteum PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009097 absent endometrial glands PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009009 absent estrous cycle PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0009009 absent estrous cycle PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009021 absent estrus PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002785 absent Leydig cells PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001132 absent mature ovarian follicles PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001155 arrest of spermatogenesis PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001155 arrest of spermatogenesis PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002286 cryptorchism PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002286 cryptorchism PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001262 decreased body weight PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005181 decreased circulating estradiol level PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005185 decreased circulating progesterone level PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002780 decreased circulating testosterone level PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002780 decreased circulating testosterone level PMID: 11145749 
Lhcgr+|Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgr+
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002780 decreased circulating testosterone level PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0009096 decreased endometrial gland number PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004929 decreased epididymis weight PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004856 decreased ovary weight PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004910 decreased seminal vesicle weight PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0004852 decreased testis weight PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004852 decreased testis weight PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002636 delayed vaginal opening PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0000642 enlarged adrenal glands PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0000691 enlarged spleen PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0003596 epididymal inflammation PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0004932 epididymis hypoplasia PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004932 epididymis hypoplasia PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009201 external male genitalia atrophy PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001926 female infertility PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001926 female infertility PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001129 impaired ovarian folliculogenesis PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001129 impaired ovarian folliculogenesis PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001260 increased body weight PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005182 increased circulating estradiol level PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001750 increased circulating follicle stimulating hormone level PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001750 increased circulating follicle stimulating hormone level PMID: 11145749 
Lhcgr+|Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgr+
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001750 increased circulating follicle stimulating hormone level PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001751 increased circulating luteinizing hormone level PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001751 increased circulating luteinizing hormone level PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0003917 increased kidney weight PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0005536 Leydig cell hypoplasia PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005536 Leydig cell hypoplasia PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0008280 male germ cell apoptosis PMID: 12604639 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001925 male infertility PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001925 male infertility PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002687 oligozoospermia PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0005147 prostate gland hypoplasia PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005147 prostate gland hypoplasia PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0003608 prostate gland inflammation PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001922 reduced male fertility PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0002254 reproductive system inflammation PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0003611 scrotum hypoplasia PMID: 11145748  15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0005148 seminal vesicle hypoplasia PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005148 seminal vesicle hypoplasia PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0004930 small epididymis PMID: 11145749  12604639 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001127 small ovary PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001127 small ovary PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0005188 small penis PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0005188 small penis PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0002774 small prostate gland PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0000664 small prostate gland anterior lobe PMID: 15514086 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001157 small seminal vesicle PMID: 15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001157 small seminal vesicle PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001153 small seminiferous tubules PMID: 11145748  15514086 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001153 small seminiferous tubules PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0001147 small testis PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0001940 testicular hypoplasia PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0008258 thin endometrium PMID: 11145749 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0008257 thin myometrium PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009088 thin uterine horn PMID: 11145748 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009081 thin uterus PMID: 11145748 
Lhcgrtm1Cvr Lhcgrtm1Cvr/Lhcgrtm1Cvr
either: 129X1/SvJ-Lhcgr or (involves: 129X1/SvJ * C57BL/6)
MGI:96783  MP:0009081 thin uterus PMID: 11145749 
Lhcgrtm1Hht Lhcgrtm1Hht/Lhcgrtm1Hht
involves: 129S7/SvEvBrd * C57BL/6J
MGI:96783  MP:0009214 vas deferens hypoplasia PMID: 15514086 
Clinically-Relevant Mutations and Pathophysiology
Disease:  Leydig cell hypoplasia, type 1
OMIM:  238320
Orphanet:  755
References: 
Mutations not determined
Disease:  Testotoxicosis
OMIM:  176410
Orphanet:  3000
Comments: 
References:  23
Mutations not determined
Disease:  Leydig cell hypoplasia due to complete LH resistance
Orphanet:  96265
References: 
Mutations not determined
Disease:  Leydig cell hypoplasia due to partial LH resistance
Orphanet:  96266
References: 
Mutations not determined
Biologically Significant Variants
Type:  Single nucleotide polymorphisms and splice variants.
Species:  Human
Description:  A number of inactivating single nucleotide polymorphisms have been identified in the human LHCGR gene, encoding (partially) inactive LH receptors.

In (genetic) males (46XY) LH receptor inactivating mutations reduce testosterone production and therefore cause absence of male sex differentiation. This condition is called 46XY pseudohermaphroditism, the patient but being genetically male with a complete set of genes (unlike true hermaphroditism where there is a defect in the sex-determining gene, SRY) but appearing female due to lack of testosterone.

In females (46XX) the presence of LH receptor inactivating mutations is less obvious as they have a normal female phenotype. However they suffer from lack of follicular maturation and ovulation, amenhorrhea, hypoestrogenicity and infertility.
References:  23
Type:  Polymorphism.
Species:  Human
Description:  A polymorphism, insLQ at position 18 in the signal peptide, has been associated with decreased survival in breast cancer patients.
References:  18
Available Assays
DiscoveRx PathHunter® CHO-K1 LHCGR β-Arrestin Cell Line (Cat no. 93-0707C2)
PathHunter® eXpress LHCGR CHO-K1 β-Arrestin GPCR Assay (Cat no. 93-0707E2CP19M)
more info

REFERENCES

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3. Channing CP, Sakai CN, Bahl OP. (1978) Role of the carbohydrate residues of human chorionic gonadotropin in binding and stimulation of adenosine 3',5'-monophosphate accumulation by porcine granulosa cells. Endocrinology103: 341-348. [PMID:217642]

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13. Minegishi T, Nakamura K, Takakura Y, Miyamoto K, Hasegawa Y, Ibuki Y, Igarashi M, Minegish T [corrected to Minegishi T. (1990) Cloning and sequencing of human LH/hCG receptor cDNA. Biochem Biophys Res Commun172: 1049-1054. [PMID:2244890]

14. Müller T, Gromoll J, Simoni M. (2003) Absence of exon 10 of the human luteinizing hormone (LH) receptor impairs LH, but not human chorionic gonadotropin action. J Clin Endocrinol Metab88: 2242-2249. [PMID:12727981]

15. Pabon JE, Bird JS, Li X, Huang ZH, Lei ZM, Sanfilippo JS, Yussman MA, Rao CV. (1996) Human skin contains luteinizing hormone/chorionic gonadotropin receptors. J Clin Endocrinol Metab81: 2738-2741. [PMID:8675605]

16. Pabon JE, Li X, Lei ZM, Sanfilippo JS, Yussman MA, Rao CV. (1996) Novel presence of luteinizing hormone/chorionic gonadotropin receptors in human adrenal glands. J Clin Endocrinol Metab81: 2397-2400. [PMID:8964884]

17. Palmer SS, Mckenna S, Arkinstall S. (2005) Discovery of new molecules for the future treatment of infertility. Reproductive Biomedicine Online 10 Suppl (Proceedings: Gonadotropins from Basic Research to Clinical Practice; Munich Germany., : 45-54.

18. Powell BL, Piersma D, Kevenaar ME, van Staveren IL, Themmen AP, Iacopetta BJ, Berns EM. (2003) Luteinizing hormone signaling and breast cancer: polymorphisms and age of onset. J Clin Endocrinol Metab88: 1653-1657. [PMID:12679452]

19. Risbridger GP, Robertson DM, de Kretser DM. (1982) The effects of chronic human chorionic gonadotropin treatment on Leydig cell function. Endocrinology110: 138-145. [PMID:6274616]

20. Rommerts FF, Cooke BA, Van der Kemp JW, van der Molen HJ. (1973) Effect of luteinizing hormone on 3',5'-cyclic AMP and testosterone production in isolated interstitial tissue of rat testis. FEBS Lett33: 114-118. [PMID:4352930]

21. Salvador LM, Maizels E, Hales DB, Miyamoto E, Yamamoto H, Hunzicker-Dunn M. (2002) Acute signaling by the LH receptor is independent of protein kinase C activation. Endocrinology143: 2986-2994. [PMID:12130564]

22. Sonoda N, Katabuchi H, Tashiro H, Ohba T, Nishimura R, Minegishi T, Okamura H. (2005) Expression of variant luteinizing hormone/chorionic gonadotropin receptors and degradation of chorionic gonadotropin in human chorionic villous macrophages. Placenta26: 298-307. [PMID:15823616]

23. Themmen APN, Huhtaniemi IT. (2000) Mutations of gonadotropins and gonadotropin receptors: elucidating the physiology and pathophysiology of pituitary-gonadal function. Endocr Rev.21: 551-583. [PMID:11041448]

24. Van Damme MP, Robertson DM, Diczfalusy E. (1974) An improved in vitro bioassay method for measuring luteinizing hormone (LH) activity using mouse Leydig cell preparations. Acta Endocrinol (Copenh)77: 655-671. [PMID:4372842]

25. van Straten NC, Schoonus-Gerritsma GG, van Someren RG, Draaijer J, Adang AE, Timmers CM, Hanssen RG, van Boeckel CA. (2002) The first orally active low molecular weight agonists for the LH receptor: thienopyr(im)idines with therapeutic potential for ovulation induction. Chembiochem3: 1023-1026. [PMID:12362369]

26. Wang C, Hsueh AJ, Erickson GF. (1981) LH stimulation of estrogen secretion by cultured rat granulosa cells. Mol Cell Endocrinol24: 17-28. [PMID:6799341]

27. Zhang FP, Poutanen M, Wilbertz J, Huhtaniemi I. (2001) Normal prenatal but arrested postnatal sexual development of luteinizing hormone receptor knockout (LuRKO) mice. Mol Endocrinol15: 172-183. [PMID:11145748]

28. Zhang M, Shi H, Segaloff DL, Van Voorhis BJ, Zheng M. (2001) Expression and localization of luteinizing hormone receptor in the female mouse reproductive tract. Biol Reprod64: 179-187. [PMID:11133673]

To cite this database page, please use the following:

Sabine Costagliola, James A. Dias, Axel P.N. Themmen, Gilbert Vassart.
Glycoprotein hormone receptors: LH receptor. Last modified on 18/03/2014. Accessed on 23/10/2014. IUPHAR database (IUPHAR-DB), http://www.iuphar-db.org/DATABASE/ObjectDisplayForward?objectId=254.

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