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In Depth Tutorials and Information
TABLE 21.1
Murine and Canine Models of Osteogenesis Imperfecta
MGI Nomenclature
a
(strain name)
b
Human OI
Classiication
c
Commercially
Available
Common Name
Gene
Genetic Background
Mouse models
Mov-13
−/−
homozygous
Col1a1
Mov13
OI type II
Col1a1
C57BL/6
No
Mov-13
/+
heterozygous
Col1a1
Mov13
(C57BL/6-
Col1a1
Mov13
/
J
)
OI type I
Col1a1
C57BL/6
Yes (Jackson
Laboratory, Bar
Harbor, ME)
Human mini-gene COL1A1
Tg(COL1A1)73Prc
OI type II-IV
Col1a1
FVB/N
No
G859C COL1A1
OI type II
Col1a1
FVB
No
Col1a1
tm1Jcm
BrtlII heterozygous
OI type II
Col1a1
129×1/SvJ * C3H/HeJ
or 129×1/SvJ * CD-1
No
BrtlIV
/+
heterozygous
Col1a1
tm1.1Jcm
OI type IV
Col1a1
129×1/SvJ * C3H/HeJ
or 129×1/SvJ * CD-1
No
Col1a1
tm1.1Jcm
BrtlIV/BrtlIV homozygous
OI type IV (very mild)
Col1a1
129×1/SvJ * C3H/HeJ
or 129×1/SvJ * CD-1
No
Col1a2
oim
(B6C3Fe
a/a-Col1a2
oim/J
)
Oim/oim
homozygous
OI type III
Col1a2
B6C3Fe a/a-
Col1a2
oim
/J, C57B6
Yes (Jackson
Laboratory)
Oim
/+
heterozygous
Col1a2
oim
(B6C3Fe
a/a-Col1a2
oim/J
)
OI type I
Col1a2
B6C3Fe a/a-
Col1a2
oim
/J,C57B6
Yes (Jackson
Laboratory)
G610C/G610C
Neo+
homozygous
Col1a2
tm1Mcbr
OI type IV
Col1a2
129/SvEv * C57BL/6J
No
+/G610C
Neo+
heterozygous
Col1a2
tm1Mcbr
OI type I/IV
Col1a2
129/SvEv * C57BL/6J
No
+/
G610C
Neo−
(Amish)
Col1a2
tm1.1Mcbr
(B6.129(FVB)-
Col1a2
tm1.1Mcbr/J
)
OI type IV
Col1a2
C57BL/6J (F1 crosses
with A/J, BALB/cByJ,
C3H/HeJ or FVB/NJ)
Yes (Jackson
Laboratory)
Aga2
−/−
homozygous
Col1a1
Aga2
OI type II
Col1a1
involves: C3HeB/FeJ
* C57BL/6J
No
Aga2
/+
heterozygous
Col1a1
Aga2
OI type III
Col1a1
involves: C3HeB/FeJ
* C57BL/6J
No
Crtap
−/−
homozygous
Crtap
tm1Brle
OI type VII
Ctrtap
involves: 129S7/
SvEvBrd
No
P3H1
−/−
, Leprecan 1 homozygous
Lepre1
tm1Dgen
OI type VIII
Lrpre1
C57BL/6
No
Ppib
tm1.1Rjb
Pbib
−/−
homozygous
OI type IX
Ppib
Not specified
No
Hsp47
−/−
Serpinh1
tm1Kzn
OI type X
Serpinh1
(involves: 129P2/
OlaHsd * C57BL/6)
or (involves: 129P2/
OlaHsd * ICR)
No
SP7
−/−
(Osx)
Osx
lox/−;
Col1a1-Cre
OI-like
Sp7
129S7/SvEvBrd *
C57BL/6 * SJL
No
OMIA number
d
Canine models
G208A COL1A1
OI type III
COL1A1
Golden retriever
No
COL1A2 frameshift
OMIA 000754-9615
OI type III
COL1A2
Beagle
No
SERPINH1 (L326P)
OMIA 001483-9615
OI type X
SERPINH1
Dachshund
No
a
Mouse Genome Informatics (MGI)/International Mouse Strain Resource (IMSR).
b
The Jackson Laboratory, Bar Harbor, Maine.
c
OI classification scheme: Bishop N. Characterising and treating osteogenesis imperfecta. Early Human Development 2010; 86(11): 743-6.
d
Online Mendelian Inheritance in Animals, OMIA, Faculty of Veterinary Science University of Sydney. World Wide Web URL:
http://omiaangisorgau/
