Posted 11 October 2010
The Tardbp gene, was isolated from a C57BL/6 genomic BAC clone (RP23-331p21; BACPAC).
Conditional Tardbp-KO mouse line generated by engineering a targeting vector in
which the 3rd exon of Tardbp was flanked by loxp together with a neomycin resistance
gene inserted in the 2nd intron; The neomycin cassette was subsequently deleted
through a crossbreeding strategy with hACTB-flp transgenic mice. The floxed Tardbp
mice were crossbred with a CAG-Cre transgenic mouse line that express the Cre recombinase
ubiquitously to generate the heterozygous Tardbp-KO (Tardbp+/-) mice.
Mutation: Conditional deletion of exon 3 of Tardbp.
Mouse strain: V26.2 C57BL/6j ES cells; injected into albino C57BL/6J; Background:
C57BL/6; Generation: F3, N3.
Tardbp+/- mice were fertile and expressed similar level of TDP-43 in a variety of
tissues compared with those of Tardbp+/+ mice. Postnatal deletion of Tardbp in mice
caused dramatic loss of body fat followed by rapid death. Moreover, conditional
Tardbp-Ko ES cells failed to proliferate.
Contact: Philip C. Wong
Department of Pathology, Division of Neuropathology
The Johns Hopkins University School of Medicine
558 Ross Research Building
720 Rutland Avenue
Baltimore, Maryland 21205-2196
E-mail: Philip C. Wong
Chiang P-M, Ling J, Jeong H-Y, Price DL, Aja SM, Wong PC. Deletion of TDP-43 down-regulates
Tbc1d1, a gene linked to obesity, and alters body fat metabolism. PNAS 107:16320,
Shan, X, Chiang P-M, Price DL and Wong, PC. Altered distribution of Gemini of coiled
bodies and mitochondria in motor neurons of TDP-43 transgenic mice. PNAS 107:16325,