Phenotypes Associated with This Genotype

Genotype
MGI:5636486

• Allelic Composition: Tg(Th-ALK*F1174L)2Loch/0; Tg(Th-MYCN)41Waw/0
• Genetic Background: involves: 129X1/SvJ * BALB/c * C57BL/6J * CBA

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

neoplasm

increased neuroblastoma incidence ( J:191012 )

• mice develop large, bulky and locally invasive thoracic and abdominal masses arising in the paraspinal ganglia or adrenals indicative of neuroblastoma

• mice show complete tumor penetrance, shorter median time to tumor onset and decreased survival at 100 days of age compared to single Tg(Th-MYCN)41Waw hemizygous mice

• tumors show decreased level of apoptosis compared to tumors from single Tg(Th-MYCN)41Waw hemizygous mice

• mice are resistant to crizotinib treatment, showing no effect on tumor size, cellularity or apoptosis

• combined treatment with crizotinib and Torin2 (an ATP-competitive inhibitor of mTOR) reduces tumor size and growth and increases apoptosis

mortality/aging

premature death ( J:191012 )

• mice show decreased survival at 100 days of age

nervous system

increased neuroblastoma incidence ( J:191012 )

• mice develop large, bulky and locally invasive thoracic and abdominal masses arising in the paraspinal ganglia or adrenals indicative of neuroblastoma

• mice show complete tumor penetrance, shorter median time to tumor onset and decreased survival at 100 days of age compared to single Tg(Th-MYCN)41Waw hemizygous mice

• tumors show decreased level of apoptosis compared to tumors from single Tg(Th-MYCN)41Waw hemizygous mice

• mice are resistant to crizotinib treatment, showing no effect on tumor size, cellularity or apoptosis

• combined treatment with crizotinib and Torin2 (an ATP-competitive inhibitor of mTOR) reduces tumor size and growth and increases apoptosis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neuroblastoma		DOID:769		J:191012