SMO

Target not currently curated in GtoImmuPdb

Target id: 239

Nomenclature: SMO

Gene and Protein Information
Previous and Unofficial Names
Database Links
Selected 3D Structures
Natural/Endogenous Ligands
Agonists
Antagonists
Allosteric Modulators
Immuno Process Associations
Transduction Mechanisms
Tissue Distribution
Expression Datasets
Functional Assays
Physiological Functions
Physiological Consequences of Altering Gene Expression
Phenotypes, Alleles and Disease Models
Clinically-Relevant Mutations and Pathophysiology
Biologically Significant Variants
References
Contributors
How to cite this page

Gene and Protein Information
Frizzled family G protein-coupled receptor
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	7	787	7q32.1	SMO	smoothened, frizzled class receptor
Mouse	7	793	6 12.36 cM	Smo	smoothened, frizzled class receptor
Rat	7	793	4q22	Smo	smoothened, frizzled class receptor

Previous and Unofficial Names
SMOH \| FZD11 \| frizzled family member 11 \| bnb \| smoothened, frizzled class receptor \| smoothened

Previous and Unofficial Names

Database Links
Alphafold	Q99835 (Hs), P56726 (Mm), P97698 (Rn)
CATH/Gene3D	1.10.2000.10
ChEMBL Target	CHEMBL5971 (Hs), CHEMBL6080 (Mm), CHEMBL1795175 (Rn)
DrugBank Target	Q99835 (Hs)
Ensembl Gene	ENSG00000128602 (Hs), ENSMUSG00000001761 (Mm), ENSRNOG00000008332 (Rn)
Entrez Gene	6608 (Hs), 319757 (Mm), 25273 (Rn)
Human Protein Atlas	ENSG00000128602 (Hs)
KEGG Gene	hsa:6608 (Hs), mmu:319757 (Mm), rno:25273 (Rn)
OMIM	601500 (Hs)
Pharos	Q99835 (Hs)
RefSeq Nucleotide	NM_005631 (Hs), NM_176996 (Mm), NM_012807 (Rn)
RefSeq Protein	NP_005622 (Hs), NP_795970 (Mm), NP_036939 (Rn)
UniProtKB	Q99835 (Hs), P56726 (Mm), P97698 (Rn)
Wikipedia	SMO (Hs)

Selected 3D Structures

Image of receptor 3D structure from RCSB PDB

Description:	Structure of the human smoothened 7TM receptor in complex with antitumor agent taladegib.
PDB Id:	4JKV
Ligand:	taladegib
Resolution:	2.45Å
Species:	Human
References:	22

Description:	Human Smoothened Receptor structure in complex with cyclopamine
PDB Id:	4O9R
Ligand:	cyclopamine
Resolution:	3.2Å
Species:	Human
References:	24

Description:	Crystal structure of a multi-domain human smoothened receptor in complex with a super stabilizing ligand, TC114
PDB Id:	5V56
Ligand:	TC114
Resolution:	2.9Å
Species:	Human
References:	26

Description:	Structure of human Smoothened-Gi complex.
PDB Id:	6OT0
Resolution:	3.84Å
Species:	Human
References:	14

Natural/Endogenous Ligands
constitutive

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Agonists

Key to terms and symbols

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Ligand		Sp.	Action	Value	Parameter	Reference
SAG1.3		Mm	Agonist	8.5	pEC₅₀	3
⤷	pEC₅₀ 8.5 (EC₅₀ 3x10^-9 M) [3] Description: Measuring Hh pathway activation in Gli-dependent reporter assays in Shh-LIGHT2 cells.
purmorphamine		Hs	Agonist	~6.0	pEC₅₀	16
⤷	pEC₅₀ ~6.0 (EC₅₀ ~1x10^-6 M) [16]

View species-specific agonist tables

Agonist Comments

Oxysterols might be endogenous agonists of the SMO receptor [4].

Antagonists

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Ligand		Sp.	Action	Value	Parameter	Reference
MRT-92		Hs	Antagonist	9.5	pK_d	6
⤷	pK_d 9.5 (K_d 3x10^-10 M) [6] Description: Binding of [³H]MRT-92 to hSMO.
sonidegib		Hs	Antagonist	8.2	pK_i	23
⤷	pK_i 8.2 (K_i 6x10^-9 M) [23]
vismodegib		Hs	Antagonist	7.8	pK_i	23
⤷	pK_i 7.8 (K_i 1.62x10^-8 M) [23]
SANT-1		Mm	Antagonist	7.7	pK_i	3
⤷	pK_i 7.7 (K_i 2x10^-8 M) [3] Description: In Gli-dependent reporter assays in Shh-LIGHT2 cells.
saridegib		Hs	Antagonist	8.9	pIC₅₀	20
⤷	pIC₅₀ 8.9 (IC₅₀ 1.4x10^-9 M) [20]
AZD8542		Mm	Antagonist	8.8	pIC₅₀	1
⤷	pIC₅₀ 8.8 (IC₅₀ 1.5x10^-9 M) [1]
AZD8542		Hs	Antagonist	8.5	pIC₅₀	1
⤷	pIC₅₀ 8.5 (IC₅₀ 3x10^-9 M) [1]
TAK-441		Mm	Antagonist	8.4	pIC₅₀	13
⤷	pIC₅₀ 8.4 (IC₅₀ 4.4x10^-9 M) [13]
glasdegib		Hs	Antagonist	8.3	pIC₅₀	12
⤷	pIC₅₀ 8.3 (IC₅₀ 5x10^-9 M) [12]
taladegib		Hs	Antagonist	8.2	pIC₅₀	8
⤷	pIC₅₀ 8.2 (IC₅₀ 6.3x10^-9 M) [8] Description: Gli-luc reporter assay.
ANTA XV		Mm	Antagonist	8.1	pIC₅₀	2
⤷	pIC₅₀ 8.1 (IC₅₀ 7.1x10^-9 M) [2] Description: Gli-luc reporter assay.
cyclopamine-KAAD		Mm	Antagonist	7.7	pIC₅₀	18
⤷	pIC₅₀ 7.7 [18] Description: Measuring Hh pathway inhibition in Gli-dependent reporter assays in Shh-LIGHT2 cells.
SANT-2		Mm	Antagonist	7.5	pIC₅₀	3
⤷	pIC₅₀ 7.5 (IC₅₀ 3x10^-8 M) [3] Description: In Gli-dependent reporter assays in Shh-LIGHT2 cells.
cyclopamine		Mm	Antagonist	~7.0	pIC₅₀	21
⤷	pIC₅₀ ~7.0 (IC₅₀ ~1x10^-7 M) [21] Description: Measuring inhibition of Hh pathway signalling in Gli-dependent reporter assays in Shh-LIGHT2 cells.

View species-specific antagonist tables

Antagonist Comments

In addition to the approved drug vismodegib, there are several more Hedgehog inhibitors (Hhi) in the drug development pipeline. Most of these compounds inhibit the hedgehog signaling pathway via antagonism of Smoothened (SMO), a key component of the pathway and include saridegib, erismodegib, BMS-833923 (XL139), TAK-441 and glasdegib (PF-04449913). SANT-3 and SANT-4 are SMO antagonists [3].

Allosteric Modulators

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Ligand											Sp.	Action	Value	Parameter	Reference
GSA-10											Hs	Positive	5.9	pEC₅₀	5
⤷	pEC₅₀ 5.9 (EC₅₀ 1.2x10^-6 M) [5] Description: Assessing differentiation of the mesenchymal pluripotent C3H10T1/2 cells into osteoblastsby measuring AP enzymatic activity as a readout for Smo agonist activity.

Immuno Process Associations

Immuno Process:

Inflammation

Primary Transduction Mechanisms
Transducer	Effector/Response
G_i/G_o family G₁₂/G₁₃ family
References: 7,15

Secondary Transduction Mechanisms
Transducer	Effector/Response
	Phospholipase A₂ stimulation
References:

Tissue Distribution

The SMO receptor is widely expressed.

Species:	Mouse
Technique:	Northern blotting.
References:	17

The SMO receptor is widely expressed.

Species:	Rat
Technique:	Northern blotting.
References:	17

Brain.

Species:	Rat
Technique:	Northern blotting.
References:	19

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

Hedgehog reporter assay in HEK 293 cells transfected with the SMO receptor and G_α12.

Species:	Human
Tissue:	HEK 293 cells.
Response measured:	Hedgehog reporter assay and Gli3 localisation.
References:	7

Measurement of IP₃ levels in HEK 293 cells transfected with the SMO receptor and G15.

Species:	Human
Tissue:	HEK 293 cells.
Response measured:	Stimulation of IP₅ production.
References:	10

Measurement of GTPγS binding in Sf9 cells transfected with the mouse SMO receptor and G proteins.

Species:	Mouse
Tissue:	Sf9 insect cells.
Response measured:	Stimulation of GTPγS binding.
References:	15

Determination of the role of GRK2 in SMO receptor functioning.

Species:	Mouse
Tissue:	C3H10T1/2 mouse cells.
Response measured:	Hedgehog/Gli reporter assay.
References:	11

Physiological Functions

SMO functions in Hedgehog signal transduction (gene targeting).

Species:	Mouse
Tissue:	Widespread.
References:	27

Physiological Consequences of Altering Gene Expression

Increasing SMO receptor expression activates Hedgehog signalling in a ligand-independent manner.

Species:	Mouse
Tissue:
Technique:	Transgenesis.
References:	18

SMO receptor knockout is embryonically lethal.

Species:	Mouse
Tissue:
Technique:	Gene targeting in embryonic stem cells.
References:	27

Phenotypes, Alleles and Disease Models

Mouse data from MGI

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Allele	Composition & genetic background	Accession	Phenotype Id	Phenotype	Reference
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0002650	abnormal ameloblast morphology	PMID: 12403705
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0004113	abnormal aortic arch morphology	PMID: 16687132
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000106	abnormal basisphenoid bone morphology	PMID: 15107405
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000822	abnormal brain ventricle morphology	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm1Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL * Swiss Webster	MGI:108075 MGI:2176172	MP:0000857	abnormal cerebellar foliation	PMID: 16571625
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000857	abnormal cerebellar foliation	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm1Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL * Swiss Webster	MGI:108075 MGI:2176172	MP:0000849	abnormal cerebellum morphology	PMID: 16571625
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000849	abnormal cerebellum morphology	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0000166	abnormal chondrocyte morphology	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0003935	abnormal craniofacial development	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000438	abnormal cranium morphology	PMID: 15107405
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0002084	abnormal developmental patterning	PMID: 12372301
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0000830	abnormal diencephalon morphology	PMID: 19854269
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002110	abnormal digit morphology	PMID: 11748145
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0004190	abnormal direction of embryo turning	PMID: 9636176
Ift172^wim\|Smo^bnb	Ift172^wim/Ift172^wim,Smo^bnb/Smo^bnb involves: C57BL/6J	MGI:108075 MGI:2682064	MP:0004252	abnormal direction of looping morphogenesis	PMID: 16061793
Smo^tm1Amc	Smo^tm1Amc/Smo^tm1Amc involves: 129X1/SvJ * C57BL/6	MGI:108075	MP:0001672	abnormal embryogenesis/ development	PMID: 17881493
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0001700	abnormal embryo turning	PMID: 12372301
Smo^tm1Amc	Smo^tm1Amc/Smo^tm1Amc involves: 129X1/SvJ * C57BL/6	MGI:108075	MP:0001700	abnormal embryo turning	PMID: 17881493
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0002576	abnormal enamel morphology	PMID: 12403705
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000100	abnormal ethmoidal bone morphology	PMID: 15107405
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0006337	abnormal first branchial arch morphology	PMID: 12372301
Smo^tm1Amc\|Tctn1^{Gt(KST296)Byg}	Tctn1^{Gt(KST296)Byg}/Tctn1^{Gt(KST296)Byg},Smo^tm1Amc/Smo^tm1Amc involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6	MGI:108075 MGI:3603820	MP:0000783	abnormal forebrain morphology	PMID: 16357211
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000841	abnormal hindbrain morphology	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0005358	abnormal incisor morphology	PMID: 12403705
Gt(ROSA)26Sor^tm6Dym\|Smo^tm2Amc\|Sox9⁺\|Sox9^tm3(cre)Crm	Gt(ROSA)26Sor^tm6Dym/?,Smo^tm2Amc/Smo^tm2Amc,Sox9^tm3(cre)Crm/Sox9⁺ involves: 129S7/SvEvBrd * 129X1/SvJ	MGI:104735 MGI:108075 MGI:98371	MP:0002932	abnormal joint morphology	PMID: 19815519
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0005271	abnormal lacrimal bone morphology	PMID: 15107405
Smo^bnb	Smo^bnb/Smo^bnb involves: C3HeB/FeJ * C57BL/6J	MGI:108075	MP:0001706	abnormal left-right axis patterning	PMID: 15755804
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0005545	abnormal lens development	PMID: 19854269
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0000269	abnormal looping morphogenesis	PMID: 9636176
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000458	abnormal mandible morphology	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004595	abnormal mandibular condyloid process morphology	PMID: 15107405
Gt(ROSA)26Sor^tm6Dym\|Smo^tm2Amc\|Sox9⁺\|Sox9^tm3(cre)Crm	Gt(ROSA)26Sor^tm6Dym/?,Smo^tm2Amc/Smo^tm2Amc,Sox9^tm3(cre)Crm/Sox9⁺ involves: 129S7/SvEvBrd * 129X1/SvJ	MGI:104735 MGI:108075 MGI:98371	MP:0004595	abnormal mandibular condyloid process morphology	PMID: 19815519
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000455	abnormal maxilla morphology	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0005587	abnormal Meckel's cartilage morphology	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0003932	abnormal molar crown morphology	PMID: 12403705
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0002689	abnormal molar morphology	PMID: 12403705
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0002239	abnormal nasal septum morphology	PMID: 15107405
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0002950	abnormal neural crest cell migration	PMID: 16687132
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0004837	abnormal neural fold formation	PMID: 12372301
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0005657	abnormal neural plate morphology	PMID: 12372301
Smo^tm1Amc\|Tulp3^hhkr	Smo^tm1Amc/Smo^tm1Amc,Tulp3^hhkr/Tulp3^hhkr involves: 129X1/SvJ * C3H/HeH * C57BL/6	MGI:108075 MGI:1329045	MP:0002151	abnormal neural tube morphology/development	PMID: 19223390
Kif7^tm1.2Hui\|Smo^tm1Amc	Kif7^tm1.2Hui/Kif7^tm1.2Hui,Smo^tm1Amc/Smo^tm1Amc involves: 129S6/SvEvTac * 129X1/SvJ * CD-1	MGI:108075 MGI:1098239	MP:0002151	abnormal neural tube morphology/development	PMID: 19549984
Kif7^tm1.2Hui\|Smo^tm1Amc	Kif7^tm1.2Hui/Kif7^tm1.2Hui,Smo^tm1Amc/Smo^tm1Amc involves: 129S6/SvEvTac * 129X1/SvJ * CD-1	MGI:108075 MGI:1098239	MP:0009937	abnormal neuron differentiation	PMID: 19549984
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0004269	abnormal optic cup morphology	PMID: 19854269
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0004268	abnormal optic stalk morphology	PMID: 19854269
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0003425	abnormal optic vesicle formation	PMID: 12372301
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0003425	abnormal optic vesicle formation	PMID: 19854269
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004457	abnormal orbitosphenoid bone morphology	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0006030	abnormal otic vesicle development	PMID: 19854269
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0006126	abnormal outflow tract development	PMID: 16687132
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000933	abnormal rhombomere morphology	PMID: 18480159
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0006355	abnormal sixth branchial arch artery morphology	PMID: 16687132
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002113	abnormal skeleton development	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002115	abnormal skeleton extremities morphology	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000104	abnormal sphenoid bone morphology	PMID: 15107405
Smo^bnb	Smo^bnb/Smo^bnb involves: C3HeB/FeJ * C57BL/6J	MGI:108075	MP:0000955	abnormal spinal cord morphology	PMID: 15755804
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004423	abnormal squamosal bone morphology	PMID: 15107405
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0006104	abnormal tectum morphology	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0005272	abnormal temporal bone morphology	PMID: 15107405
Olig3⁺\|Olig3^tm1(cre)Ynka\|Smo^tm2Amc	Olig3^tm1(cre)Ynka/Olig3⁺,Smo^tm2Amc/Smo^tm2Amc involves: 129X1/SvJ * C57BL/6J	MGI:108075 MGI:2149955	MP:0000832	abnormal thalamus morphology	PMID: 19357274
Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm2Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129X1/SvJ * C57BL/6 * SJL	MGI:108075 MGI:2176172	MP:0000832	abnormal thalamus morphology	PMID: 19357274
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0002260	abnormal thyroid cartilage morphology	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0002100	abnormal tooth morphology	PMID: 15107405
Smo^tm1Amc	Smo^tm1Amc/Smo^tm1Amc involves: 129X1/SvJ * C57BL/6	MGI:108075	MP:0003229	abnormal vitelline vasculature morphology	PMID: 17881493
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0008381	absent gonial bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004318	absent incus	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004791	absent lower incisors	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004319	absent malleus	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000040	absent middle ear ossicles	PMID: 15107405
Kif7^tm1.2Hui\|Smo^tm1Amc	Kif7^tm1.2Hui/Kif7^tm1.2Hui,Smo^tm1Amc/Smo^tm1Amc involves: 129S6/SvEvTac * 129X1/SvJ * CD-1	MGI:108075 MGI:1098239	MP:0004949	absent neuronal precursor cells	PMID: 19549984
Smo^tm1Amc	Smo^tm1Amc/Smo^tm1Amc involves: 129X1/SvJ * CD-1	MGI:108075	MP:0004949	absent neuronal precursor cells	PMID: 19549984
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004476	absent palatine bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004449	absent presphenoid bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004454	absent pterygoid process	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004916	absent Reichert cartilage	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004204	absent stapes	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0008386	absent styloid process	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0009905	absent tongue	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0003138	absent tympanic ring	PMID: 15107405
Bmp4⁺\|Bmp4^tm2Blh\|Smo^tm1Amc	Bmp4^tm2Blh/Bmp4⁺,Smo^tm1Amc/Smo^tm1Amc involves: 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:88180	MP:0001719	absent vitelline blood vessels	PMID: 17881493
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0000099	absent vomer bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0001293	anophthalmia	PMID: 19854269
Smo^tm2Amc\|Tg(Gfap-cre)73.12Mvs	Smo^tm2Amc/Smo^tm2Amc,Tg(Gfap-cre)73.12Mvs/0 involves: 129X1/SvJ * BALB/c * C57BL/6NHsd	MGI:108075 MGI:3522207	MP:0003354	astrocytosis	PMID: 20943901
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0010403	atrial septal defect	PMID: 16687132
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0004574	broad limb buds	PMID: 9636176
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm1Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL * Swiss Webster	MGI:108075 MGI:2176172	MP:0000774	decreased brain size	PMID: 16571625
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0001698	decreased embryo size	PMID: 12372301
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0001698	decreased embryo size	PMID: 9636176
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0001698	decreased embryo size	PMID: 19854269
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0004686	decreased length of long bones	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)15Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)15Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176259	MP:0004686	decreased length of long bones	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)3Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)3Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176260	MP:0004686	decreased length of long bones	PMID: 11748145
Ift172^wim\|Smo^bnb	Ift172^wim/Ift172^wim,Smo^bnb/Smo^bnb involves: C57BL/6J	MGI:108075 MGI:2682064	MP:0000939	decreased motor neuron number	PMID: 16061793
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0000292	distended pericardium	PMID: 12372301
Smo^tm1Amc	Smo^tm1Amc/Smo^tm1Amc involves: 129X1/SvJ * CD-1	MGI:108075	MP:0003984	embryonic growth retardation	PMID: 19549984
Smo^bnb	Smo^bnb/Smo^bnb involves: C3HeB/FeJ * C57BL/6J	MGI:108075	MP:0006207	embryonic lethality during organogenesis	PMID: 15755804
Ift172^wim\|Smo^bnb	Ift172^wim/Ift172^wim,Smo^bnb/Smo^bnb involves: C57BL/6J	MGI:108075 MGI:2682064	MP:0006207	embryonic lethality during organogenesis	PMID: 16061793
Kif7^maki\|Smo^bnb\|Tg(Hlxb9-GFP)1Tmj	Kif7^maki/Kif7^maki,Smo^bnb/Smo^bnb,Tg(Hlxb9-GFP)1Tmj/? involves: C57BL/6J * FVB/N	MGI:108075 MGI:1098239 MGI:3037037	MP:0006207	embryonic lethality during organogenesis	PMID: 19666503
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0009048	enlarged tectum	PMID: 18480159
Kif7^tm1.2Hui\|Smo^tm1Amc	Kif7^tm1.2Hui/Kif7^tm1.2Hui,Smo^tm1Amc/Smo^tm1Amc involves: 129S6/SvEvTac * 129X1/SvJ * CD-1	MGI:108075 MGI:1098239	MP:0000914	exencephaly	PMID: 19549984
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Fgf15-cre)1Hisa	Smo^tm1Amc/Smo^tm2Amc,Tg(Fgf15-cre)1Hisa/0 involves: 129X1/SvJ * Swiss Webster	MGI:108075 MGI:4430280	MP:0000784	forebrain hypoplasia	PMID: 19854269
Smo^tm1.1Pab	Smo^tm1.1Pab/Smo^tm1.1Pab Not Specified	MGI:108075	MP:0010335	fused first branchial arch	PMID: 12372301
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0001914	hemorrhage	PMID: 9636176
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0001701	incomplete embryo turning	PMID: 9636176
Smo^tm1Amc\|Tulp3^hhkr	Smo^tm1Amc/Smo^tm1Amc,Tulp3^hhkr/Tulp3^hhkr involves: 129X1/SvJ * C3H/HeH * C57BL/6	MGI:108075 MGI:1329045	MP:0002809	increased spinal cord size	PMID: 19223390
Smo^tm1Amc\|Smo^tm2Amc\|Tg(KRT14-cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(KRT14-cre)1Amc/0 involves: 129X1/SvJ * C57BL/6 * CBA	MGI:108075 MGI:2450263	MP:0002058	neonatal lethality	PMID: 12403705
Smo^tm2Amc\|Tg(mI56i-cre,EGFP)1Kc	Smo^tm2Amc/Smo^tm2Amc,Tg(mI56i-cre,EGFP)1Kc/? involves: 129X1/SvJ	MGI:108075 MGI:3609984	MP:0002169	no abnormal phenotype detected	PMID: 16221724
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Sp7-tTA,tetO-EGFP/cre)1Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0 involves: 129X1/SvJ * CD-1	MGI:108075 MGI:3689349	MP:0002169	no abnormal phenotype detected	PMID: 16854976
Ift172^wim\|Smo^bnb	Ift172^wim/Ift172^wim,Smo^bnb/Smo^bnb involves: C57BL/6J	MGI:108075 MGI:2682064	MP:0000929	open neural tube	PMID: 16061793
Smo^bnb	Smo^bnb/Smo^bnb involves: C3H/HeJ * C57BL/6J	MGI:108075	MP:0001787	pericardial edema	PMID: 9636176
Smo^tm1Amc\|Tulp3^hhkr	Smo^tm1Amc/Smo^tm1Amc,Tulp3^hhkr/Tulp3^hhkr involves: 129X1/SvJ * C3H/HeH * C57BL/6	MGI:108075 MGI:1329045	MP:0001787	pericardial edema	PMID: 19223390
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0002081	perinatal lethality	PMID: 16687132
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0002633	persistent truncus arteriosis	PMID: 16687132
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002082	postnatal lethality	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm1Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL * Swiss Webster	MGI:108075 MGI:2176172	MP:0002082	postnatal lethality	PMID: 16571625
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0004158	right aortic arch	PMID: 16687132
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002765	short fibula	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0004351	short humerus	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0004340	short scapula	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0002764	short tibia	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Col2a1-cre)10Amc	Smo^tm1Amc/Smo^tm2Amc,Tg(Col2a1-cre)10Amc/0 involves: 129X1/SvJ	MGI:108075 MGI:2176261	MP:0004359	short ulna	PMID: 11748145
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0003108	short zygomatic bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004459	small alisphenoid bone	PMID: 15107405
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Nes-cre)1Kln	Smo^tm1Amc/Smo^tm2Amc,Tg(Nes-cre)1Kln/0 involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL * Swiss Webster	MGI:108075 MGI:2176172	MP:0000852	small cerebellum	PMID: 16571625
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000852	small cerebellum	PMID: 18480159
Smo^tm1Amc\|Smo^tm2Amc\|Tg(Wnt1-cre)11Rth	Smo^tm1Amc/Smo^tm2Amc,Tg(Wnt1-cre)11Rth/? involves: C57BL/6J * CBA/J	MGI:108075 MGI:2447280	MP:0004470	small nasal bone	PMID: 15107405
En1^tm2(cre)Wrst\|Gli3^tm1Alj\|Smo^tm2Amc	En1^tm2(cre)Wrst/?,Gli3^tm1Alj/Gli3^tm1Alj,Smo^tm2Amc/Smo^tm2Amc involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6	MGI:108075 MGI:95389 MGI:95729	MP:0000873	thin external granule cell layer	PMID: 18480159
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0004110	transposition of great arteries	PMID: 16687132
Isl1⁺\|Isl1^tm1(cre)Sev\|Smo^tm1Amc\|Smo^tm2Amc	Isl1^tm1(cre)Sev/Isl1⁺,Smo^tm1Amc/Smo^tm2Amc involves: 129X1/SvJ	MGI:101791 MGI:108075	MP:0010402	ventricular septal defect	PMID: 16687132

Clinically-Relevant Mutations and Pathophysiology

Disease:

Basal cell carcinoma

Disease Ontology:

DOID:2513

References:

Click column headers to sort

Type	Species	Amino acid change	Nucleotide change	Description	Reference
Missense	Human	W535L		Activating mutation	25
Missense	Human	R562Q		Activating mutation	25

Biologically Significant Variants

Type:	Point mutation
Species:	Mouse
Description:	An oncogenic point mutation has been identified in the mouse Smo gene.
References:	9

References

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1. AstraZeneca. AZD8542. Accessed on 12/09/2014. Modified on 12/09/2014. astrazeneca.com, http://openinnovation.astrazeneca.com/what-we-offer/compound/azd8542/

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3. Chen JK, Taipale J, Young KE, Maiti T, Beachy PA. (2002) Small molecule modulation of Smoothened activity. Proc Natl Acad Sci USA, 99 (22): 14071-6. [PMID:12391318]

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6. Hoch L, Faure H, Roudaut H, Schoenfelder A, Mann A, Girard N, Bihannic L, Ayrault O, Petricci E, Taddei M et al.. (2015) MRT-92 inhibits Hedgehog signaling by blocking overlapping binding sites in the transmembrane domain of the Smoothened receptor. FASEB J, 29 (5): 1817-29. [PMID:25636740]

7. Kasai K, Takahashi M, Osumi N, Sinnarajah S, Takeo T, Ikeda H, Kehrl JH, Itoh G, Arnheiter H. (2004) The G12 family of heterotrimeric G proteins and Rho GTPase mediate Sonic hedgehog signalling. Genes Cells, 9 (1): 49-58. [PMID:14723707]

8. Lu X, Peng Y, Wang C, Yang J, Bao X, Dong Q, Zhao W, Tan W, Dong X. (2017) Design, synthesis, and biological evaluation of optimized phthalazine derivatives as hedgehog signaling pathway inhibitors. Eur J Med Chem, 138: 384-395. [PMID:28688278]

9. Mao J, Ligon KL, Rakhlin EY, Thayer SP, Bronson RT, Rowitch D, McMahon AP. (2006) A novel somatic mouse model to survey tumorigenic potential applied to the Hedgehog pathway. Cancer Res, 66 (20): 10171-8. [PMID:17047082]

10. Masdeu C, Faure H, Coulombe J, Schoenfelder A, Mann A, Brabet I, Pin JP, Traiffort E, Ruat M. (2006) Identification and characterization of Hedgehog modulator properties after functional coupling of Smoothened to G15. Biochem Biophys Res Commun, 349 (2): 471-9. [PMID:16945339]

11. Meloni AR, Fralish GB, Kelly P, Salahpour A, Chen JK, Wechsler-Reya RJ, Lefkowitz RJ, Caron MG. (2006) Smoothened signal transduction is promoted by G protein-coupled receptor kinase 2. Mol Cell Biol, 26 (20): 7550-60. [PMID:16908539]

12. Munchhof MJ, Li Q, Shavnya A, Borzillo GV, Boyden TL, Jones CS, LaGreca SD, Martinez-Alsina L, Patel N, Pelletier K et al.. (2012) Discovery of PF-04449913, a Potent and Orally Bioavailable Inhibitor of Smoothened. ACS Med Chem Lett, 3 (2): 106-11. [PMID:24900436]

13. Ohashi T, Oguro Y, Tanaka T, Shiokawa Z, Tanaka Y, Shibata S, Sato Y, Yamakawa H, Hattori H, Yamamoto Y et al.. (2012) Discovery of the investigational drug TAK-441, a pyrrolo[3,2-c]pyridine derivative, as a highly potent and orally active hedgehog signaling inhibitor: modification of the core skeleton for improved solubility. Bioorg Med Chem, 20 (18): 5507-17. [PMID:22898254]

14. Qi X, Liu H, Thompson B, McDonald J, Zhang C, Li X. (2019) Cryo-EM structure of oxysterol-bound human Smoothened coupled to a heterotrimeric G_i. Nature, 571 (7764): 279-283. [PMID:31168089]

15. Riobo NA, Saucy B, Dilizio C, Manning DR. (2006) Activation of heterotrimeric G proteins by Smoothened. Proc Natl Acad Sci USA, 103 (33): 12607-12. [PMID:16885213]

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18. Taipale J, Chen JK, Cooper MK, Wang B, Mann RK, Milenkovic L, Scott MP, Beachy PA. (2000) Effects of oncogenic mutations in Smoothened and Patched can be reversed by cyclopamine. Nature, 406 (6799): 1005-9. [PMID:10984056]

19. Traiffort E, Charytoniuk DA, Faure H, Ruat M. (1998) Regional distribution of Sonic Hedgehog, patched, and smoothened mRNA in the adult rat brain. J Neurochem, 70 (3): 1327-30. [PMID:9489757]

20. Tremblay MR, Lescarbeau A, Grogan MJ, Tan E, Lin G, Austad BC, Yu LC, Behnke ML, Nair SJ, Hagel M et al.. (2009) Discovery of a potent and orally active hedgehog pathway antagonist (IPI-926). J Med Chem, 52 (14): 4400-18. [PMID:19522463]

21. Turner MW, Cruz R, Mattos J, Baughman N, Elwell J, Fothergill J, Nielsen A, Brookhouse J, Bartlett A, Malek P et al.. (2016) Cyclopamine bioactivity by extraction method from Veratrum californicum. Bioorg Med Chem, 24 (16): 3752-7. [PMID:27338657]

22. Wang C, Wu H, Katritch V, Han GW, Huang XP, Liu W, Siu FY, Roth BL, Cherezov V, Stevens RC. (2013) Structure of the human smoothened receptor bound to an antitumour agent. Nature, 497 (7449): 338-43. [PMID:23636324]

23. Wang J, Mook Jr RA, Lu J, Gooden DM, Ribeiro A, Guo A, Barak LS, Lyerly HK, Chen W. (2012) Identification of a novel Smoothened antagonist that potently suppresses Hedgehog signaling. Bioorg Med Chem, 20 (22): 6751-7. [PMID:23063522]

24. Weierstall U, James D, Wang C, White TA, Wang D, Liu W, Spence JC, Bruce Doak R, Nelson G, Fromme P et al.. (2014) Lipidic cubic phase injector facilitates membrane protein serial femtosecond crystallography. Nat Commun, 5: 3309. [PMID:24525480]

25. Xie J, Murone M, Luoh SM, Ryan A, Gu Q, Zhang C, Bonifas JM, Lam CW, Hynes M, Goddard A et al.. (1998) Activating Smoothened mutations in sporadic basal-cell carcinoma. Nature, 391 (6662): 90-2. [PMID:9422511]

26. Zhang X, Zhao F, Wu Y, Yang J, Han GW, Zhao S, Ishchenko A, Ye L, Lin X, Ding K et al.. (2017) Crystal structure of a multi-domain human smoothened receptor in complex with a super stabilizing ligand. Nat Commun, 8: 15383. [PMID:28513578]

27. Zhang XM, Ramalho-Santos M, McMahon AP. (2001) Smoothened mutants reveal redundant roles for Shh and Ihh signaling including regulation of L/R asymmetry by the mouse node. Cell, 105 (6): 781-92. [PMID:11440720]

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