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Tg(Thy1-SERPINI1*G392E)333Icka
Transgene Detail
Summary
Symbol: Tg(Thy1-SERPINI1*G392E)333Icka
Name: transgene insertion 333, Ichiro Kato
MGI ID: MGI:3833390
Synonyms: G392E-Tg
Transgene: Tg(Thy1-SERPINI1*G392E)333Icka  Location: unknown  
Alliance: Tg(Thy1-SERPINI1*G392E)333Icka page
Transgene
origin
Strain of Origin:  (C57BL x DBA)F1
Transgene
description
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
 
Tg(Thy1-SERPINI1*G392E)333Icka expresses 1 gene
 
Mutation detailsThe promoter was used to drive neuron-specific expression of the human cDNA containing nucleotide substitutions that result in the amino acid substitution of glutamic acid for glycine at position 392 (G392E). This mutation was originally discovered in patients with familial encephalopathy with neuroserpin inclusion bodies (FENIB). Three lines were created (333, 42, 115) with line 333 expressing at much higher levels than lines 42 and 115. (J:144649)
Phenotypes
Key:
hm homozygous ht heterozygous tg involves transgenes phenotype observed
cn conditional genotype  cx complex: > 1 genome feature ot other: hemizygous, indeterminate,... N normal phenotype
Genotype/
Background:
Allelic Composition
Genetic Background
Cell Line(s)
tg1  Disease Model
involves: C57BL * CD-1 * DBA
 
tg2  Disease Model
involves: C57BL * CD-1 * DBA
 
Phenotypes:
Affected Systems
show or hide all annotated terms
   
behavior/neurological
increased susceptibility to pharmacologically induced seizures
nervous system
increased susceptibility to pharmacologically induced seizures
abnormal neuron morphology
View phenotypes and curated references for all genotypes (concatenated display).
Disease models
Key:
disease model   expected model not found
Models:
Human Diseases
tg1
tg2
IDs
Find Mice (IMSR)
Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 0 strains available      Cell Lines: 0 lines available
References
Original:  J:144649 Takasawa A, et al., Mutation-, aging-, and gene dosage-dependent accumulation of neuroserpin (G392E) in endoplasmic reticula and lysosomes of neurons in transgenic mice. J Biol Chem. 2008 Dec 19;283(51):35606-13
All:  1 reference(s)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
12/17/2024
MGI 6.24
The Jackson Laboratory

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