All Phenotypes Tg(BGLAP-cre)1Clem MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Tg(BGLAP-cre)1Clem
transgene insertion 1, Thomas L Clemens
MGI:2446069

Summary

24 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Phex^tm1.1Mkd/Y Tg(BGLAP-cre)1Clem/0	B6.Cg-Phex^tm1.1Mkd Tg(BGLAP-cre)1Clem	MGI:3775086
cn2	Rb1^tm2Brn/Rb1^tm2Brn Trp53^tm1Brn/Trp53^tm1Brn Tg(BGLAP-cre)1Clem/0	involves: 129 * 129P2/OlaHsd * FVB/NJ	MGI:7482566
cn3	Dicer1^tm1Mmk/Dicer1^tm1Mmk Tg(BGLAP-cre)1Clem/0	involves: 129 * C57BL/6 * FVB/N	MGI:4452406
cn4	Ptch1^tm1Yy/Ptch1⁺ Tg(BGLAP-cre)1Clem/0	involves: 129 * C57BL/6 * FVB/NJ	MGI:5781000
cn5	Ptch1^tm1Yy/Ptch1^tm1Yy Tg(BGLAP-cre)1Clem/0	involves: 129 * C57BL/6 * FVB/NJ	MGI:5780999
cn6	Igf1r^tm2Arge/Igf1r^tm2Arge Tg(BGLAP-cre)1Clem/0	involves: 129 * FVB/N	MGI:2651814
cn7	Nell1^tm1Kuv/Nell1^tm1Kuv Rb1^tm2Brn/Rb1^tm2Brn Trp53^tm1Brn/Trp53^tm1Brn Tg(BGLAP-cre)1Clem/0	involves: 129 * FVB/NJ	MGI:7482567
cn8	Grem1^tm1Ecan/Grem1^tm1Rmh Tg(BGLAP-cre)1Clem/0	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N	MGI:3761936
cn9	Cxcl12^tm1Tng/Cxcl12^tm1.1Link Tg(BGLAP-cre)1Clem/0	involves: 129P2/OlaHsd * C57BL/6J * C57BL/6NTac * FVB/N	MGI:5468940
cn10	Tnfsf11^tm1.1Caob/Tnfsf11^tm1.1Caob Tg(BGLAP-cre)1Clem/0	involves: 129S * 129X1/SvJ * C57BL/6 * FVB/NJ	MGI:5297378
cn11	Amer1^tm1.1Nbar/Y Tg(BGLAP-cre)1Clem/0	involves: 129S4/SvJae * C57BL/6 * FVB/NJ * SJL	MGI:5086011
cn12	Ccn2^tm1Mga/Ccn2^tm1.1Vlcg Tg(BGLAP-cre)1Clem/0	involves: 129S6/SvEvTac * C57BL/6NTac * FVB/NJ	MGI:4822058
cn13	Ptch1^tm1Yy/Ptch1⁺ Trp53^tm1Brd/Trp53⁺ Tg(BGLAP-cre)1Clem/0	involves: 129S7/SvEvBrd * C57BL/6 * FVB/NJ	MGI:5781001
cn14	Bglap/Bglap2^tm1Kry/Bglap⁺ Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: 129S7/SvEvBrd * FVB/NJ	MGI:6198739
cn15	Bglap/Bglap2^tm1Kry/Bglap2⁺ Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: 129S7/SvEvBrd * FVB/NJ	MGI:6198741
cn16	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/? Tg(BGLAP-cre)1Clem/? Trip11^tm1.1Psmi/Trip11^tm1.2Psmi	involves: 129/Sv * C57BL/6 * FVB/NJ	MGI:6154164
cn17	Clstn3^{tm1c(EUCOMM)Hmgu}/Clstn3^{tm1c(EUCOMM)Hmgu} Tg(BGLAP-cre)1Clem/0	involves: C57BL/6 * C57BL/6N * FVB/NJ * SJL	MGI:6454085
cn18	Notch2^tm2.1Ecan/Notch2^tm2.1Ecan Tg(BGLAP-cre)1Clem/0	involves: C57BL/6J * FVB/NJ	MGI:6157651
cn19	Il11^tm2.1Moto/Il11^tm2.1Moto Tg(BGLAP-cre)1Clem/0	involves: C57BL/6NCrlj * CBA/JNCrlj * FVB/NJ	MGI:7659221
cn20	Prmt5^{tm2c(EUCOMM)Wtsi}/Prmt5^{tm2c(EUCOMM)Wtsi} Tg(BGLAP-cre)1Clem/0	involves: C57BL/6N * FVB/NJ	MGI:7277817
cn21	Vkorc1^tm1.1Kry/Vkorc1^tm1.1Kry Vkorc1l1^tm1.1Kry/Vkorc1l1^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: FVB/NJ	MGI:6198758
cn22	Vkorc1l1^tm1.1Kry/Vkorc1l1^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: FVB/NJ	MGI:6198746
cn23	Vkorc1^tm1.1Kry/Vkorc1^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: FVB/NJ	MGI:6198744
cn24	Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0	involves: FVB/NJ	MGI:6198737

Allelic Composition	Phex^tm1.1Mkd/Y Tg(BGLAP-cre)1Clem/0
Genetic Background	B6.Cg-Phex^tm1.1Mkd Tg(BGLAP-cre)1Clem

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Phex^tm1.1Mkd mutation (0 available); any Phex mutation (22 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

decreased circulating phosphate level ( J:131043 )

• similar to the decrease in Phex^hyp hemizygous male mice at 8 weeks of age

abnormal renal phosphate reabsorption ( J:131043 )

• reduction in renal phosphate uptake at 8 weeks of age is similar to that in Phex^hyp hemizygous male mice

renal/urinary system

abnormal renal phosphate reabsorption ( J:131043 )

• reduction in renal phosphate uptake at 8 weeks of age is similar to that in Phex^hyp hemizygous male mice

skeleton

decreased length of long bones ( J:131043 )

short femur ( J:131043 )

abnormal osteocyte lacunocanalicular system morphology ( J:131043 )

• the inner lacunocanalicular wall is buckled and enlarged

abnormal osteocyte lacuna morphology ( J:131043 )

• osteocyte lacunae are increased in size and randomly organized compared to those in control bone

osteomalacia ( J:131043 )

• osteomalacia characterized by hyperosteoidosis and an excess of unmineralilized osteoid

• severity of osteomalacia is similar to that of Phex^hyp hemizygous male mice

limbs/digits/tail

short femur ( J:131043 )

Allelic Composition	Rb1^tm2Brn/Rb1^tm2Brn Trp53^tm1Brn/Trp53^tm1Brn Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * 129P2/OlaHsd * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rb1^tm2Brn mutation (3 available); any Rb1 mutation (111 available)
Tg(BGLAP-cre)1Clem mutation (1 available)
Trp53^tm1Brn mutation (18 available); any Trp53 mutation (240 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

decreased tumor-free survival time ( J:326989 )

• tumor-free survival is 146.5 days

neoplasm

increased osteosarcoma incidence ( J:326989 )

• 52.4% incidence

skeleton

increased osteosarcoma incidence ( J:326989 )

• 52.4% incidence

Allelic Composition	Dicer1^tm1Mmk/Dicer1^tm1Mmk Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dicer1^tm1Mmk mutation (0 available); any Dicer1 mutation (96 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

abnormal bone structure ( J:158901 )

• texture of mineralized is altered compared to in wild-type mice

increased compact bone volume ( J:158901 )

• cortical bone volume is increased compared to in wild-type mice

hematopoietic system

hematopoietic system phenotype ( J:158901 )

N	• mice exhibit normal hematopoietic morphology

Allelic Composition	Ptch1^tm1Yy/Ptch1⁺ Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * C57BL/6 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptch1^tm1Yy mutation (0 available); any Ptch1 mutation (115 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

neoplasm

neoplasm ( J:214349 )

N	• mice do not exhibit any bone tumor formation up to 16 months of age

mortality/aging

mortality/aging ( J:214349 )

N	• normal survival rate

Allelic Composition	Ptch1^tm1Yy/Ptch1^tm1Yy Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * C57BL/6 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptch1^tm1Yy mutation (0 available); any Ptch1 mutation (115 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

premature death ( J:214349 )

• mice die within 3 months of age

skeleton

abnormal bone ossification ( J:214349 )

• focal bone overgrowth is frequently seen in long bones

Allelic Composition	Igf1r^tm2Arge/Igf1r^tm2Arge Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Igf1r^tm2Arge mutation (1 available); any Igf1r mutation (88 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

abnormal skeleton morphology ( J:80190 )

• phenotype is quantitatively similar, but less pronounced in males compared to females

thin neurocranium ( J:80190 )

• calvarial cortical thickness is reduced to ~80% of control

decreased osteoclast cell number ( J:80190 )

• significant reduction in number (67-69%) per bone perimeter is seen relative to controls

decreased osteoblast cell number ( J:80190 )

• significant reduction in number (67-69%) per bone perimeter is seen relative to controls

abnormal trabecular bone morphology ( J:80190 )

• trabecular separation is increased by 44% compared to controls

• in secondary spongiosa of distal femur in 6-week old females, trabecular separation is increased; trabecular connectivity is 35% lower than normal

decreased trabecular bone volume ( J:80190 )

• trabecular bone volume is decreased by 46%

• in secondary spongiosa of distal femur in 6-week old females, cancellous bone volume is ~24% lower than controls

decreased bone trabecula number ( J:80190 )

• trabecular number is decreased by 29%

decreased trabecular bone thickness ( J:80190 )

• trabecular thickness is decreased by 25%

abnormal skeleton physiology ( J:80190 )

• phenotype is quantitatively similar, but less pronounced in males compared to females

abnormal osteoblast physiology ( J:80190 )

• increase in osteoclast erosion surface accompanies increase in osteoid

abnormal osteoclast physiology ( J:80190 )

• amount of osteoid is significantly increased in female mutants at 6-weeks

decreased bone mineralization ( J:80190 )

• mineral apposition rate is impaired in 6-week old females

decreased bone ossification ( J:80190 )

• in 3-week old females, epiphyseal bone formation rate is reduced to ~42% of control mice

craniofacial

thin neurocranium ( J:80190 )

• calvarial cortical thickness is reduced to ~80% of control

immune system

decreased osteoclast cell number ( J:80190 )

• significant reduction in number (67-69%) per bone perimeter is seen relative to controls

abnormal osteoclast physiology ( J:80190 )

• amount of osteoid is significantly increased in female mutants at 6-weeks

hematopoietic system

decreased osteoclast cell number ( J:80190 )

• significant reduction in number (67-69%) per bone perimeter is seen relative to controls

abnormal osteoclast physiology ( J:80190 )

• amount of osteoid is significantly increased in female mutants at 6-weeks

cellular

abnormal osteoblast physiology ( J:80190 )

• increase in osteoclast erosion surface accompanies increase in osteoid

Allelic Composition	Nell1^tm1Kuv/Nell1^tm1Kuv Rb1^tm2Brn/Rb1^tm2Brn Trp53^tm1Brn/Trp53^tm1Brn Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nell1^tm1Kuv mutation (1 available); any Nell1 mutation (57 available)
Rb1^tm2Brn mutation (3 available); any Rb1 mutation (111 available)
Tg(BGLAP-cre)1Clem mutation (1 available)
Trp53^tm1Brn mutation (18 available); any Trp53 mutation (240 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

increased tumor-free survival time ( J:326989 )

• tumor-free survival is 398 days compared to 146.5 days in mutant mice wild-type for Nell1

neoplasm

abnormal tumor morphology ( J:326989 )

• 85.2% decrease in the proliferative-index in tumors compared to tumors in mutant mice wild-type for Nell1

abnormal tumor vascular morphology ( J:326989 )

• 66.1% decrease in tumor-associated vasculature compared to mutant mice wild-type for Nell1

decreased osteosarcoma incidence ( J:326989 )

• 15.8% incidence compared to 52.4% incidence in mutant mice wild-type for Nell1

skeleton

decreased osteosarcoma incidence ( J:326989 )

• 15.8% incidence compared to 52.4% incidence in mutant mice wild-type for Nell1

Allelic Composition	Grem1^tm1Ecan/Grem1^tm1Rmh Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grem1^tm1Ecan mutation (0 available); any Grem1 mutation (19 available)
Grem1^tm1Rmh mutation (1 available); any Grem1 mutation (19 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

enhanced osteoblast differentiation ( J:126375 )

• in vitro osteoblast maturation is enhanced

increased trabecular bone volume ( J:126375 )

• 40% increase in trabecular bone volume due to increased bone thickness

increased trabecular bone thickness ( J:126375 )

increased bone mineralization ( J:126375 )

• the mineral apposition rate is enhanced

increased bone ossification ( J:126375 )

• bone formation rate is 2.5 fold higher than in littermate controls

cellular

enhanced osteoblast differentiation ( J:126375 )

• in vitro osteoblast maturation is enhanced

Allelic Composition	Cxcl12^tm1Tng/Cxcl12^tm1.1Link Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6J * C57BL/6NTac * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxcl12^tm1.1Link mutation (1 available); any Cxcl12 mutation (25 available)
Cxcl12^tm1Tng mutation (1 available); any Cxcl12 mutation (25 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hematopoietic system

hematopoietic system phenotype ( J:193594 )

N	• mice exhibit normal hematopoietic stem cell and lymphoid progenitors morphology and physiology

decreased B cell number ( J:193594 )

• in the blood

immune system

decreased B cell number ( J:193594 )

• in the blood

Allelic Composition	Tnfsf11^tm1.1Caob/Tnfsf11^tm1.1Caob Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S * 129X1/SvJ * C57BL/6 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(BGLAP-cre)1Clem mutation (1 available)
Tnfsf11^tm1.1Caob mutation (1 available); any Tnfsf11 mutation (32 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

failure of tooth eruption ( J:177558 )

• in some mice

increased bone mineral density ( J:177558 )

• at 5 weeks of age

increased trabecular bone volume ( J:177558 )

• at 5 weeks of age

abnormal cartilage morphology ( J:177558 )

• mice exhibit unresorbed cartilage unlike wild-type mice

increased long bone epiphyseal plate size ( J:177558 )

• widened growth plate in the femur

craniofacial

failure of tooth eruption ( J:177558 )

• in some mice

growth/size/body

failure of tooth eruption ( J:177558 )

• in some mice

Allelic Composition	Amer1^tm1.1Nbar/Y Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S4/SvJae * C57BL/6 * FVB/NJ * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Amer1^tm1.1Nbar mutation (0 available); any Amer1 mutation (2 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

skeleton phenotype ( J:173242 )

N	• no gross skeletal defects are detected

Allelic Composition	Ccn2^tm1Mga/Ccn2^tm1.1Vlcg Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S6/SvEvTac * C57BL/6NTac * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ccn2^tm1.1Vlcg mutation (0 available); any Ccn2 mutation (29 available)
Ccn2^tm1Mga mutation (0 available); any Ccn2 mutation (29 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

decreased bone mineral density ( J:163027 )

• at 1 month of age the phenotype is similar to that in Ctgf^tm1Mga single homozygotes

• at 1 month of age osteopenia is seen

• at 6 months of age males show a decrease in bone volume secondary to a decrease in trabecular number

abnormal trabecular bone morphology ( J:163027 )

• at 1 month and 6 months of age the number of trabeculae is reduced

Allelic Composition	Ptch1^tm1Yy/Ptch1⁺ Trp53^tm1Brd/Trp53⁺ Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptch1^tm1Yy mutation (0 available); any Ptch1 mutation (115 available)
Tg(BGLAP-cre)1Clem mutation (1 available)
Trp53^tm1Brd mutation (5 available); any Trp53 mutation (240 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

paralysis ( J:214349 )

• lower body paralysis due to spine tumors

neoplasm

increased metastatic potential ( J:214349 )

• occasionally, pulmonary metastasis is seen in the lungs

increased osteosarcoma incidence ( J:214349 )

• mice develop bone tumors as early as 7 months of age, with incidence significantly increased at 11 months onwards with about 70% penetrance

• bone tumors are located mostly in the forelimbs and hindlimbs and frequently in the spine and are found less frequently in the ribs and skull

• primary tumors are composed of highly mineralized tissues and abundant osteoids with multinucleated cells resembling human osteoblastic osteosarcoma

• the majority of mutants develop only bone tumors

skeleton

increased osteosarcoma incidence ( J:214349 )

• mice develop bone tumors as early as 7 months of age, with incidence significantly increased at 11 months onwards with about 70% penetrance

• bone tumors are located mostly in the forelimbs and hindlimbs and frequently in the spine and are found less frequently in the ribs and skull

• primary tumors are composed of highly mineralized tissues and abundant osteoids with multinucleated cells resembling human osteoblastic osteosarcoma

• the majority of mutants develop only bone tumors

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
osteosarcoma		DOID:3347	OMIM:259500	J:214349

Allelic Composition	Bglap/Bglap2^tm1Kry/Bglap⁺ Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S7/SvEvBrd * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bglap/Bglap2^tm1Kry mutation (1 available); any Bglap mutation (13 available)
Ggcx^tm1.1Kry mutation (0 available); any Ggcx mutation (37 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:264734 )

N	• glucose tolerance is normal

Allelic Composition	Bglap/Bglap2^tm1Kry/Bglap2⁺ Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: 129S7/SvEvBrd * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bglap/Bglap2^tm1Kry mutation (1 available); any Bglap2 mutation (9 available)
Ggcx^tm1.1Kry mutation (0 available); any Ggcx mutation (37 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:264734 )

N	• glucose tolerance is normal

Allelic Composition	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/? Tg(BGLAP-cre)1Clem/? Trip11^tm1.1Psmi/Trip11^tm1.2Psmi
Genetic Background	involves: 129/Sv * C57BL/6 * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo} mutation (10 available); any Gt(ROSA)26Sor mutation (993 available)
Tg(BGLAP-cre)1Clem mutation (1 available)
Trip11^tm1.1Psmi mutation (1 available); any Trip11 mutation (99 available)
Trip11^tm1.2Psmi mutation (0 available); any Trip11 mutation (99 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

cellular

abnormal Golgi stack morphology ( J:253969 )

• humeral osteoblasts have no swelling of the endoplasmic reticulum, but do have an abnormal Golgi apparatus stack structure

skeleton

skeleton phenotype ( J:253969 )

• despite the osteoblast-specific disruption of a gene invovled in vesicle transport, these mice develop normally with both endochondral and intramembranous bones being of normal size and mineralization, and the osteoblasts having no swelling of the endoplasmic reticulum, although the osteoblast Golgi apparatus stack structure is not normal, and at 6 weeks of age micro CT measurements are normal

Allelic Composition	Clstn3^{tm1c(EUCOMM)Hmgu}/Clstn3^{tm1c(EUCOMM)Hmgu} Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: C57BL/6 * C57BL/6N * FVB/NJ * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Clstn3^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Clstn3 mutation (66 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

limbs/digits/tail phenotype ( J:293885 )

N	• normal bone morphology

Allelic Composition	Notch2^tm2.1Ecan/Notch2^tm2.1Ecan Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: C57BL/6J * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch2^tm2.1Ecan mutation (0 available); any Notch2 mutation (99 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

increased osteoclast cell number ( J:246017 )

• distal femurs exhibit an increase in osteoclast numbers

• however, the number of osteoblasts and bone formation rates are not different from controls

abnormal femur morphology ( J:246017 )

• distal femurs exhibit an increase in eroded surface

decreased bone mineral density ( J:246017 )

• cancellous and cortical bone osteopenia is seen in females, but not males, at 1 month of age

• a modest cortical osteopenia with cortical thinning and increased porosity is seen in both males and females at 4 months of age

abnormal compact bone morphology ( J:246017 )

• cortical bone porosity is increased in females at 1 and 4 months of age and in males at 4 months of age

decreased compact bone volume ( J:246017 )

• females exhibit decreased cortical bone volume in the femoral midshaft at 1 and 4 months of age, while males show a decrease at 4 months of age

decreased compact bone area ( J:246017 )

• cortical bone area is decreased in 1 month old females

decreased compact bone thickness ( J:246017 )

• cortical bone thickness is decreased in females at 1 and 4 months of age and in males at 4 months of age

abnormal trabecular bone morphology ( J:246017 )

decreased trabecular bone volume ( J:246017 )

• females exhibit decreased trabecular bone volume in the distal femur at 1 and 4 months of age, with 1 month old females showing an approximate 50% reduction in cancellous bone volume secondary to a reduced number of trabeculae and connectivity density

• at 4 months of age, the skeletal phenotype of females is less pronounced, and cancellous bone volume/total volume is 30% lower than in controls

abnormal bone trabecula morphology ( J:246017 )

• connectivity density is lower and structure model index is higher, indicating a prevalence of rod-like trabeculae over plate-like trabeculae

decreased bone trabecula number ( J:246017 )

• females exhibit decreased trabecular number in the distal femur at 1 and 4 months of age

increased bone trabecular spacing ( J:246017 )

• females exhibit increased trabecular separation in the distal femur at 1 and 4 months of age

decreased trabecular bone connectivity density ( J:246017 )

• 1 month old females exhibit reduced connectivity density

decreased trabecular bone thickness ( J:246017 )

• females exhibit decreased trabecular thickness in the distal femur at 4 months of age

decreased bone mineralization ( J:246017 )

• cortical bone shows suppressed endocortical mineral apposition rate

hematopoietic system

increased osteoclast cell number ( J:246017 )

• distal femurs exhibit an increase in osteoclast numbers

• however, the number of osteoblasts and bone formation rates are not different from controls

immune system

increased osteoclast cell number ( J:246017 )

• distal femurs exhibit an increase in osteoclast numbers

• however, the number of osteoblasts and bone formation rates are not different from controls

limbs/digits/tail

abnormal femur morphology ( J:246017 )

• distal femurs exhibit an increase in eroded surface

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Hajdu-Cheney syndrome		DOID:2736	OMIM:102500	J:246017

Allelic Composition	Il11^tm2.1Moto/Il11^tm2.1Moto Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: C57BL/6NCrlj * CBA/JNCrlj * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Il11^tm2.1Moto mutation (0 available); any Il11 mutation (21 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

adipose tissue

increased white adipose tissue amount ( J:345337 )

• adipose tissue area is increased in mice fed either regular diet or high-fat diet but is larger in mice under high-fat diet than under regular diet

• treatment with WAY-262611, a beta-catenin agonist and Dkk1 inhibitor, reduces adipose tissue area

homeostasis/metabolism

decreased circulating osteocalcin level ( J:345337 )

• serum osteocalcin levels are reduced

abnormal circulating interleukin level ( J:345337 )

• serum IL-11 levels are reduced

impaired glucose tolerance ( J:345337 )

• mice develop glucose intolerance, showing increased blood glucose after glucose loading by oral glucose tolerance test under both regular diet and high-fat diet

insulin resistance ( J:345337 )

• mice develop insulin resistance, showing increased fasting level of serum insulin in an oral glucose tolerance test

• homeostasis model assessment of insulin resistance (HOMA-IR) is increased under regular diet and high-fat diet

immune system

abnormal circulating interleukin level ( J:345337 )

• serum IL-11 levels are reduced

skeleton

decreased bone mineral density ( J:345337 )

• decrease in cortical and cancellous bone mineral density

decreased bone mass ( J:345337 )

abnormal ossification involved in bone remodeling ( J:345337 )

• while mechanical unloading reduces vertebral cancellous bone marrow density as in controls, mechanical reloading shows a lower increase in bone marrow density than seen in controls

Allelic Composition	Prmt5^{tm2c(EUCOMM)Wtsi}/Prmt5^{tm2c(EUCOMM)Wtsi} Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: C57BL/6N * FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prmt5^{tm2c(EUCOMM)Wtsi} mutation (1 available); any Prmt5 mutation (45 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

skeleton

skeleton phenotype ( J:283043 )

N	• no signs of scoliosis at P10 or 2 months of age

Allelic Composition	Vkorc1^tm1.1Kry/Vkorc1^tm1.1Kry Vkorc1l1^tm1.1Kry/Vkorc1l1^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(BGLAP-cre)1Clem mutation (1 available)
Vkorc1l1^tm1.1Kry mutation (0 available); any Vkorc1l1 mutation (22 available)
Vkorc1^tm1.1Kry mutation (0 available); any Vkorc1 mutation (7 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

abnormal circulating osteocalcin level ( J:264734 )

• increase in GLU-OCN levels as in mice with wild-type Vkorc1l1

Allelic Composition	Vkorc1l1^tm1.1Kry/Vkorc1l1^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(BGLAP-cre)1Clem mutation (1 available)
Vkorc1l1^tm1.1Kry mutation (0 available); any Vkorc1l1 mutation (22 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:264734 )

N	• mice exhibit normal circulating levels of GLA-, GLU- and total OCN levels

Allelic Composition	Vkorc1^tm1.1Kry/Vkorc1^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(BGLAP-cre)1Clem mutation (1 available)
Vkorc1^tm1.1Kry mutation (0 available); any Vkorc1 mutation (7 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:264734 )

N	• slight but not significant improvement in glucose tolerance

abnormal circulating osteocalcin level ( J:264734 )

• decreased GLA-OCN, further upon prevention of coprophagy

• 10-fold increase in GLU-OCN levels

• however, total OCN levels are normal

hematopoietic system

hematopoietic system phenotype ( J:264734 )

N	• unlike in null mice, no hemostatic defects were detected

Allelic Composition	Ggcx^tm1.1Kry/Ggcx^tm1.1Kry Tg(BGLAP-cre)1Clem/0
Genetic Background	involves: FVB/NJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ggcx^tm1.1Kry mutation (0 available); any Ggcx mutation (37 available)
Tg(BGLAP-cre)1Clem mutation (1 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

reproductive system

reproductive system phenotype ( J:264734 )

N	• mice do not exhibit hypergonadism with normal sperm count, testis weight, epididymis weight, and seminal vesicle weight

homeostasis/metabolism

decreased circulating osteocalcin level ( J:264734 )

• GLA-OCN and total OCN levels decreased 75 and 10 times, respectively

improved glucose tolerance ( J:264734 )

skeleton

skeleton phenotype ( J:264734 )

N	• mice exhibit normal trabecular and cortical bone density, numbers of osteoblast and osteoblasts, bone formation rates and osteoclastic activity

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