All Phenotypes Mib1<tm2Kong> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Mib1^tm2Kong
targeted mutation 1, Young-Yun Kong
MGI:3804448

Summary

5 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Mib1^tm2Kong/Mib1^tm2Kong Tg(Tek-cre)12Flv/0	involves: 129P2/OlaHsd * C3H * C57BL/6	MGI:3804815
cn2	Mib1^tm2Kong/Mib1^tm2Kong Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * C57BL/6	MGI:3804816
cn3	Mib1^em1Jlp/Mib1^tm2Kong Tg(Tnnt2-cre)5Blh/0	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:7544909
cn4	Mib1^tm2Kong/Mib1^tm2Kong Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:3804817
cn5	Mib1^tm2Kong/Mib1^tm2Kong Foxa2^{tm3.1(icre)Heli}/Foxa2⁺	involves: 129P2/OlaHsd * C57BL/6 * SJL	MGI:5428765

Allelic Composition	Mib1^tm2Kong/Mib1^tm2Kong Tg(Tek-cre)12Flv/0
Genetic Background	involves: 129P2/OlaHsd * C3H * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)
Tg(Tek-cre)12Flv mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:130369 )

• mice die at around E11.5

embryo

absent vitelline blood vessels ( J:130369 )

• at E9.5 and E10.5

embryonic growth retardation ( J:130369 )

• at E9.5 and E10.5

abnormal vitelline vascular remodeling ( J:130369 )

• at E9.5 and E10.5, mice exhibit defects characteristic of vascular remodeling

cardiovascular system

cardiovascular system phenotype ( J:130369 )

N	• despite defects in vascular remodeling, vasculogenesis is normal

abnormal dorsal aorta morphology ( J:130369 )

• the dorsal aorta is narrower than in wild-type mice and arterial specification is abnormal

abnormal vascular regression ( J:130369 )

• capillary networks are less extensive and more primitive than in wild-type mice

absent vitelline blood vessels ( J:130369 )

• at E9.5 and E10.5

pericardial effusion ( J:130369 )

• at E9.5 and E10.5

hematopoietic system

abnormal definitive hematopoiesis ( J:137915 )

• cultures of para-aortic splanchnopleura produce fewer colony forming cells when cultured alone or on OP9 cells compared to cultured wild-type cells

growth/size/body

embryonic growth retardation ( J:130369 )

• at E9.5 and E10.5

homeostasis/metabolism

pericardial effusion ( J:130369 )

• at E9.5 and E10.5

cellular

abnormal vascular regression ( J:130369 )

• capillary networks are less extensive and more primitive than in wild-type mice

Allelic Composition	Mib1^tm2Kong/Mib1^tm2Kong Tg(Msx2-cre)5Rem/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)
Tg(Msx2-cre)5Rem mutation (2 available)

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

limbs/digits/tail

syndactyly ( J:130369 )

fused phalanges ( J:130369 )

• digits 2 through 4 are fused in adulthood

• mice exhibit fusion of soft tissues and nails of digits 2 and 3

abnormal digit development ( J:130369 )

• at E13.5, the interdigit space between digits 2 and 3 is narrower than in wild-type mice

abnormal interdigital cell death ( J:130369 )

• at E15.5, interdigit separation is absent due to a decrease in interdigital cell death

growth/size/body

epidermal cyst ( J:130369 )

• at P28, mice exhibit epidermal cysts below the interfollicular epidermis that are filled with cornified materials

decreased body size ( J:130369 )

skeleton

fused phalanges ( J:130369 )

• digits 2 through 4 are fused in adulthood

• mice exhibit fusion of soft tissues and nails of digits 2 and 3

integument

hairless ( J:130369 )

• mice are bald at 4 weeks of age

premature hair loss ( J:130369 )

• mice begin to lose their hair along the dorsal midline and bald at 4 weeks of age

decreased hair follicle number ( J:130369 )

• at 28 days, hair follicles are barely detected in the back skin

abnormal epidermal layer morphology ( J:130369 )

• at P28, the epidermal cell layer has numerous squame

epidermal cyst ( J:130369 )

• at P28, mice exhibit epidermal cysts below the interfollicular epidermis that are filled with cornified materials

thick epidermis ( J:130369 )

• at P28

cellular

abnormal interdigital cell death ( J:130369 )

• at E15.5, interdigit separation is absent due to a decrease in interdigital cell death

Allelic Composition	Mib1^em1Jlp/Mib1^tm2Kong Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1^em1Jlp mutation (0 available); any Mib1 mutation (55 available)
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

abnormal trabecula carnea morphology ( J:338889 )

• noncompacted trabeculae in ventricular myocardium in adults

thin left ventricle myocardium compact layer ( J:338889 )

• left ventricular compact layer 45% thinner in E16.5 embryos

• reduced compact-to-trabecular myocardium ratio in E16.5 embryos

abnormal cardiac outflow tract development ( J:338889 )

• various conotruncal defects in E16.5 embryos

abnormal aortic valve morphology ( J:338889 )

• dysplasia in adults

bicuspid aortic valve ( J:338889 )

• in E16.5 embryos

dilated heart ventricle ( J:338889 )

• in adults

cardiac fibrosis ( J:338889 )

• myocardial fibrosis in septum and around coronary vessels in adults

decreased cardiac muscle contractility ( J:338889 )

• reduced fractional shortening and ejection fraction in adults

aortic valve regurgitation ( J:338889 )

• in adults

mortality/aging

lethality during fetal growth through weaning, incomplete penetrance ( J:338889 )

• only 5% of embryos develop to adulthood

muscle

abnormal trabecula carnea morphology ( J:338889 )

• noncompacted trabeculae in ventricular myocardium in adults

thin left ventricle myocardium compact layer ( J:338889 )

• left ventricular compact layer 45% thinner in E16.5 embryos

• reduced compact-to-trabecular myocardium ratio in E16.5 embryos

decreased cardiac muscle contractility ( J:338889 )

• reduced fractional shortening and ejection fraction in adults

Allelic Composition	Mib1^tm2Kong/Mib1^tm2Kong Tg(GFAP-cre)25Mes/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)
Tg(GFAP-cre)25Mes mutation (2 available)

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

mortality/aging

premature death ( J:130369 )

• mice die at 2 to 4 months of age

nervous system

delayed neuronal migration ( J:130369 )

• mice exhibit a delay in the migration of cells from the external granule layer to the internal granule layer

reduced cerebellar foliation ( J:130369 )

• reduced at P9

abnormal cerebellum external granule cell layer morphology ( J:130369 )

• at P9, the external granule cell layer contains more granule cells than in wild-type mice

• unlike in wild-type mice, granule cells are maintained at P17 but migrate to the internal granule cell layer by P21

• however, cell proliferation within the external granule layer is normal

small cerebellum ( J:130369 )

• at P9

abnormal CNS glial cell morphology ( J:130369 )

• at P15, Bergmann glial cell processes are severely disorganized

growth/size/body

decreased body size ( J:130369 )

cellular

delayed neuronal migration ( J:130369 )

• mice exhibit a delay in the migration of cells from the external granule layer to the internal granule layer

Allelic Composition	Mib1^tm2Kong/Mib1^tm2Kong Foxa2^{tm3.1(icre)Heli}/Foxa2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Foxa2^{tm3.1(icre)Heli} mutation (0 available); any Foxa2 mutation (28 available)
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)

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

endocrine/exocrine glands

absent pancreatic duct ( J:184814 )

• loss of DBA+ve (D. biflorus agglutinin) duct structures is observed by E18.5

abnormal pancreas development ( J:184814 )

• at E9.5, an excess of Neurog3+ve cells is found in the dorsal pancreatic bud; near complete conversion of dorsal bud cells to glucagon+ve cells occurs by E10.5

• at E10.5, dorsal pancreas is absent, but ventral pancreatic anlage forms normally

• proximal-distal patterning is altered in the embryonic pancreas; at E15.5, Nkx6-1+ve, Ptf1a-ve cells are absent from the proximal domain while Hnf1b and DBA+ve cells are also lost, with a concurrent increase in Nkx6-1-ve, Ptf1a+ve cells as well as Cpa+ve and amylase +ve cells

• at E12.5, Nkx6-1+ve Ptf1a-ve are present, but reduced in numbers by 50% compared to controls along with a corresponding increase in Nkx6-1-ve Ptf1a+ve cells

abnormal pancreatic beta cell differentiation ( J:184814 )

• insulin-producing cells are absent by E18.5

digestive/alimentary system

absent pancreatic duct ( J:184814 )

• loss of DBA+ve (D. biflorus agglutinin) duct structures is observed by E18.5

cellular

abnormal pancreatic beta cell differentiation ( J:184814 )

• insulin-producing cells are absent by E18.5

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