Tg(SOD1*G93A)1Gur
Transgene Detail

Summary

• Symbol: Tg(SOD1*G93A)1Gur
• Name: transgene insertion 1, Mark E Gurney
• MGI ID: MGI:2183719
• Synonyms: G1H, G93A, G93A+, G93A SOD1, G93A-SOD1, (G93A)Tg+, Gur1-G93A, hSOD1G93A, SOD1 G93A, SOD1^G93A, SOD1 Tg, Tg(G93A-SOD1)1Gur, TgN(SOD1-G93A)1Gur, TgN[SOD1-G93A]1Gur, Tg(SOD1-G93A)1Gur
• Transgene: Tg(SOD1*G93A)1Gur Location: Chr12:97132574-97132574 bp Genetic Position: Chr12, cytoband E
• Alliance: Tg(SOD1*G93A)1Gur page

Presence of neurofilament spheroids in the spinal cord of an 82-day old Tg(SOD1)2Gur/0 mouse

Show the 5 phenotype image(s) involving this allele.

Transgene origin

• Strain of Origin: (C57BL/6 x SJL)F1

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(SOD1*G93A)1Gur expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: This transgenic subline (designated G1H in J:76718) is derived from the G1 parental transgenic line (originally described in J:32665). This line carries a 40% expansion in transgene copy number compared to the original G1 line (described in J:32665, in MGI as Tg(SOD1*G93A)2Gur). The transgene construct is composed of the human SOD1 gene carrying a glycine to alanine transition at position 93 (G93A). The G93A mutation does not alter the activity of the protein. This line carries a high copy number maps to Mus Chr12:97,165,800 (coordinates from MGSC ver 37, mm9). (J:32665, J:76718)

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)

	Allelic Composition	Genetic Background	Cell Line(s)
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cn1	Gt(ROSA)26Sor^tm1(GRN)Pvd/Gt(ROSA)26Sor^tm1(GRN)Pvd Tg(Pgk1-cre)1Lni/0 Tg(SOD1*G93A)1Gur/0	B6J.Cg-Tg(Pgk1-cre)1Lni Gt(ROSA)26Sor^tm1(GRN)Pvd Tg(SOD1*G93A)1Gur
cn2	Gt(ROSA)26Sor^tm1(GRN)Pvd/Gt(ROSA)26Sor^+ Tg(Pgk1-cre)1Lni/0 Tg(SOD1*G93A)1Gur/0	B6J.Cg-Tg(Pgk1-cre)1Lni Gt(ROSA)26Sor^tm1(GRN)Pvd Tg(SOD1*G93A)1Gur
cx3	Gria2^tm1Rod/Gria2^+ Tg(SOD1*G93A)1Gur/0	B6.Cg-Gria2^tm1Rod Tg(SOD1*G93A)1Gur
cx4	Gria2^tm1Rod/Gria2^tm1Rod Tg(SOD1*G93A)1Gur/0	B6.Cg-Gria2^tm1Rod Tg(SOD1*G93A)1Gur
cx5	Tcrb^tm1Mom/Tcrb^+ Tg(SOD1*G93A)1Gur/0	B6.Cg-Tcrb^tm1Mom Tg(SOD1*G93A)1Gur/J
cx6	Sirt2^tm1.2Auw/Sirt2^tm1.2Auw Tg(SOD1*G93A)1Gur/0	involves: 129 * C57BL/6 * C57BL/6J * SJL
cx7	Hdac6^tm1.1Pmt/Y Tg(SOD1*G93A)1Gur/0	involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * SJL
cx8	Slc1a2^tm1Kta/Slc1a2^+ Tg(SOD1*G93A)1Gur/0	involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * SJL/J
cx9	Cx3cr1^tm1Litt/Cx3cr1^tm1Litt Tg(SOD1*G93A)1Gur/0	involves: 129P2/OlaHsd * C57BL/6 * SJL
cx10	Cnr1^tm1Map/Cnr1^tm1Map Tg(SOD1*G93A)1Gur/0	involves: 129S1/Sv * 129X1/SvJ * ABH * C57BL/6 * SJL
cx11	Tg(SOD1*G93A)1Gur/0 Vegfa^tm2Pec/Vegfa^tm2Pec	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
cx12	Spi1^tm1Ram/Spi1^tm1Ram Tg(SOD1*G93A)1Gur/0	involves: 129S2/SvPas * C57BL/6 * SJL
cx13	Fgfbp1^tm1Gvdz/Fgfbp1^tm1Gvdz Tg(SOD1*G93A)1Gur/0 Tg(Thy1-YFP)16Jrs/0	involves: 129S5/SvEvBrd * C57BL/6J * CBA * SJL
cx14	Mmp9^tm1Tvu/Mmp9^tm1Tvu Tg(SOD1*G93A)1Gur/0	involves: 129S6/SvEvTac * C57BL/6 * CD-1 * SJL
cx15	Tg(SOD1*G93A)1Gur/0 Tg(THY1-SNCA)1Sud/0	involves: 129S6/SvEvTac * C57BL/6 * SJL
cx16	Als2^tm1Deng/Als2^tm1Deng Tg(SOD1*G93A)1Gur/?	involves: 129/Sv * C57BL/6 * SJL
cx17	Faah^tm1Crv/Faah^tm1Crv Tg(SOD1*G93A)1Gur/0	involves: 129X1/SvJ * ABH * C57BL/6 * SJL
cx18	Dctn1^tm1Cai/Dctn1^+ Tg(SOD1*G93A)1Gur/0	involves: 129X1/SvJ * C57BL/6 * FVB/N * SJL
cx19	Bax^tm1Sjk/Bax^tm1Sjk Tg(SOD1*G93A)1Gur/0	involves: 129X1/SvJ * C57BL/6 * SJL
cx20	Dync1h1^Cra1/Dync1h1^+ Tg(SOD1*G93A)1Gur/?	involves: C3HeB/FeJ * C57BL/6 * SJL
cx21	Sod2^tm1Cje/Sod2^+ Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * CD-1 * SJL
cx22	Tg(Myl1-Igf1)1Nros/0 Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * FVB/N * SJL
cx23	Tg(Gria2*586N)238.2Rsp/0 Tg(SOD1*G93A)1Gur/0	involves: C57BL/6Ico
cx24	Tg(SOD1*G93A)1Gur/0 Wld^s/+	involves: C57BL/6J * C57BL/6Ola * SJL/J
cx25	Tg(SOD1*G93A)1Gur/0 Wld^s/Wld^s	involves: C57BL/6J * C57BL/6Ola * SJL/J
cx26	Bbc3^tm1Ast/Bbc3^tm1Ast Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * NZB * SJL
cx27  Disease Model	Tg(SOD1)2Gur/0 Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * SJL
tg28  Disease Model	Tg(SOD1*G93A)1Gur/0	B6.Cg-Tg(SOD1*G93A)1Gur
tg29  Disease Model	Tg(SOD1*G93A)1Gur/0	B6.Cg-Tg(SOD1*G93A)1Gur/J
tg30  Disease Model	Tg(SOD1*G93A)1Gur/0	B6SJL-Tg(SOD1*G93A)1Gur
tg31  Disease Model	Tg(SOD1*G93A)1Gur/0	B6SJL-Tg(SOD1*G93A)1Gur/J
tg32	Tg(SOD1*G93A)1Gur/0	involves: ABH * C57BL/6 * SJL
tg33	Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * CD-1 * SJL
tg34	Tg(SOD1*G93A)1Gur/0	involves: C57BL/6Ico
tg35	Tg(SOD1*G93A)1Gur/0	involves: C57BL/6J * C57BL/Ola * SJL/J
tg36  Disease Model	Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * SJL
tg37	Tg(SOD1*G93A)1Gur/0	SJL.Cg-Tg(SOD1*G93A)1Gur
tg38	Tg(SOD1*G93A)1Gur/?	involves: C57BL/6 * SJL

Phenotypes:

Affected Systems	cn1	cn2	cx3	cx4	cx5	cx6	cx7	cx8	cx9	cx10	cx11	cx12	cx13	cx14	cx15	cx16	cx17	cx18	cx19	cx20	cx21	cx22	cx23	cx24	cx25	cx26	cx27	tg28	tg29	tg30	tg31	tg32	tg33	tg34	tg35	tg36	tg37	tg38
show or hide all annotated terms Sex:
adipose tissue																																				√
abnormal adipose tissue morphology																																				√
abnormal fat pad morphology																																				√
behavior/neurological	√	√		√			√		√		√							√	√	√	√		√			√		√	√	√	√			√		√		√
behavior/neurological phenotype																										N
abnormal active avoidance behavior																																				√
abnormal drinking behavior																															√
increased anxiety-related response																															√
abnormal fear-related response																														√
abnormal motor capabilities/coordination/movement																															√					√
impaired righting response																							√
tremors																							√					√	√		√			√
impaired coordination	√	√		√							√								√		√							√	√		√					√
decreased grip strength				√			√		√																	√		√			√					√
abnormal locomotor behavior																																				√
abnormal gait																													√							√
long stride length																													√
short stride length																																				√
decreased vertical activity																												√
decreased locomotor activity																				√								√			√							√
paralysis																		√																		√
hindlimb paralysis				√																											√					√
cardiovascular system																															√
abnormal vascular endothelial cell morphology																															√
abnormal capillary morphology																															√
cellular								√											√											√	√					√
abnormal endoplasmic reticulum morphology																															√
abnormal mitochondrial morphology																			√											√	√
abnormal mitochondrial crista morphology																															√
abnormal mitochondrial shape																															√
increased mitochondrial size																															√
dilated mitochondrion																															√
abnormal cell physiology								√																							√					√
increased cellular sensitivity to oxidative stress																																				√
decreased cellular glucose uptake																																				√
abnormal mitochondrial physiology																															√
abnormal respiratory electron transport chain																															√
growth/size/body					√				√			N								√			√			√		√			√			√		√		√
growth/size/body region phenotype												N
decreased body weight																				√								√								√		√
slow postnatal weight gain																															√
weight loss					√																		√			√								√		√
increased susceptibility to weight loss									√
hematopoietic system					√																								√		√
increased basophil cell number																															√
decreased leukocyte cell number																															√
decreased eosinophil cell number																															√
decreased monocyte cell number																															√
abnormal lymphocyte morphology																															√
decreased lymphocyte cell number																															√
abnormal microglial cell morphology																															√
microgliosis																															√
abnormal spleen morphology																													√		√
small spleen																															√
decreased spleen weight																													√		√
abnormal splenic cell ratio																															√
abnormal splenocyte morphology																															√
decreased splenocyte number																													√
abnormal T cell physiology																													√
abnormal microglial cell physiology					√
homeostasis/metabolism																								√							√					√
abnormal circulating hormone level																																				√
increased circulating corticosterone level																																				√
decreased circulating insulin level																																				√
decreased circulating leptin level																																				√
abnormal energy expenditure																																				√
edema																															√
abnormal nitric oxide homeostasis																																				√
increased oxygen consumption																																				√
abnormal glucose homeostasis																															√
abnormal calcium ion homeostasis																															√
slow Wallerian degeneration																								√
increased cerebral infarct size																																				√
immune system					√									√															√		√
increased basophil cell number																															√
decreased leukocyte cell number																															√
decreased eosinophil cell number																															√
decreased monocyte cell number																															√
abnormal lymphocyte morphology																															√
decreased lymphocyte cell number																															√
abnormal microglial cell morphology																															√
microgliosis																															√
abnormal spleen morphology																													√		√
small spleen																															√
decreased spleen weight																													√		√
abnormal splenic cell ratio																															√
abnormal splenocyte morphology																															√
decreased splenocyte number																													√
abnormal T cell physiology																													√
abnormal microglial cell physiology					√
decreased tumor necrosis factor secretion														√
CNS inflammation																															√
mortality/aging	√	√		√	√	√	√	√	√	√	√	√	√	N		√	√		√	√	√	√	√	√	√	√	√	√	√		√	√		√	√	√	√	√
mortality/aging														N
abnormal survival							√
extended life span										√
premature death	√	√		√	√	√		√	√		√	√	√			√	√		√	√	√	√	√	√	√	√	√	√	√		√	√		√	√	√	√	√
muscle											√						√					√						√	√		√					√
abnormal skeletal muscle fiber morphology																															√
decreased skeletal muscle fiber diameter																						√							√
centrally nucleated skeletal muscle fibers																						√
abnormal skeletal muscle fiber type ratio																						√							√
decreased skeletal muscle size																												√
skeletal muscle atrophy																																				√
abnormal muscle physiology																	√														√					√
muscle weakness											√																				√					√
progressive muscle weakness																															√
nervous system	√	√	√	√	√	√	√	√	√			√	√	N	√	√	√		√	√	√	√		√		√	√		√	√	√		√		√	√		√
nervous system phenotype														N
abnormal microglial cell morphology																															√
abnormal brain morphology																															√
abnormal brainstem morphology																															√
abnormal midbrain morphology																															√
abnormal superior colliculus morphology																															√
abnormal oculomotor nucleus morphology																															√
abnormal substantia nigra morphology																															√
decreased substantia nigra size																					√												√
abnormal tegmentum morphology																															√
abnormal red nucleus morphology																															√
abnormal globus pallidus morphology																															√
abnormal hypothalamus morphology																															√
abnormal thalamus morphology																															√
abnormal cerebral cortex morphology																															√
brain vacuoles																															√
abnormal astrocyte morphology																															√
gliosis																			√							√										√
microgliosis																															√
astrocytosis																				√		√									√					√		√
abnormal innervation pattern to muscle						√	√
abnormal corticospinal tract morphology																															√
abnormal neuron morphology																														√
abnormal cholinergic neuron morphology																														√
abnormal synaptic bouton morphology																															√
abnormal dendrite morphology																														√
abnormal dendritic spine morphology																														√
decreased neuron number						√			√												√												√
decreased sensory neuron number																								√											√
increased neuron number							√
abnormal neuromuscular synapse morphology													√
abnormal spinal cord morphology																				√										√	√					√		√
abnormal motor neuron morphology																			√		√									√	√					√
abnormal motor neuron innervation pattern																			√					√
decreased motor neuron number			√	√				√				√							√	√		√		√					√	√	√					√		√
motor neuron degeneration	√	√			√							√					√			√						√			√	√	√					√
abnormal spinal cord interneuron morphology																														√
abnormal ventral interneuron 1 morphology																														√
abnormal spinal cord ventral horn morphology																														√	√
decreased spinal cord ventral horn cell number																					√												√
abnormal spinal cord white matter morphology																														√
decreased spinal cord size																					√
neurodegeneration															√	√
abnormal nervous system physiology																											√				√					√
abnormal microglial cell physiology					√
increased cerebral infarct size																																				√
CNS inflammation																															√
abnormal action potential						√	√
abnormal neuron physiology																																				√
slow Wallerian degeneration																								√
abnormal axonal transport																															√
impaired synaptic plasticity																																				√
abnormal CNS synaptic transmission																																				√
abnormal excitatory postsynaptic potential																																				√
abnormal AMPA-mediated synaptic currents				√
abnormal long-term potentiation																																				√
reduced long-term depression																																				√
abnormal paired-pulse inhibition																																				√

View phenotypes and curated references for all genotypes (concatenated display).

Disease models

Key:

√

disease model

expected model not found

Models:

Human Diseases	cx27	tg28	tg29	tg30	tg31	tg36
amyotrophic lateral sclerosis type 1 IDs amyotrophic lateral sclerosis type 1       DOID:0060193 ICD10CM:G12.2 OMIM:105400 Close	√	√	√	√	√	√

Expression

In Structures Affected by this Mutation:

29 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 4 strains available Cell Lines: 0 lines available

Notes

This line, G1H, was derived from the original G1 line (now designated Tg(SOD1*G93A)2Gur) reported in J:32665.

Transgenic mice on a background that involves C57BL/6 and SJL express high levels of the transgene with a 4-fold increase in SOD activity, and exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans. Hemizygous transgenic mice become paralyzed in one or more limbs and have a life span of approximately 19-23 weeks. Paralysis is due to loss of motor neurons from the spinal cord.

References

• Original: J:76718 Tu PH, et al., Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A. 1996 Apr 2;93(7):3155-60
• All: 1133 reference(s)