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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Thy1-YFP)HJrs
transgene insertion H, Joshua R Sanes
MGI:3497947
Summary 19 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Cmtr1tm1b(EUCOMM)Hmgu/Cmtr1tm1b(EUCOMM)Hmgu
Emx1tm1(cre)Krj/Emx1+
Tg(Thy1-YFP)HJrs/0
involves: 129S2/SvPas * C57BL/6J * C57BL/6N * CBA MGI:6502664
cn2
Ntrk2tm2Lfr/Ntrk2tm2Lfr
Tg(Six3-cre)69Frty/?
Tg(Thy1-YFP)HJrs/?
involves: C57BL/6 * CBA * DBA/2 MGI:3717378
cx3
Cttnbp2em1Yph/Cttnbp2+
Tg(Thy1-YFP)HJrs/0
B6.Cg-Cttnbp2em1Yph Tg(Thy1-YFP)HJrs MGI:6694271
cx4
Cttnbp2em1Yph/Cttnbp2em1Yph
Tg(Thy1-YFP)HJrs/0
B6.Cg-Cttnbp2em1Yph Tg(Thy1-YFP)HJrs MGI:6694268
cx5
Cttnbp2em2Yph/Cttnbp2+
Tg(Thy1-YFP)HJrs/0
B6.Cg-Cttnbp2em2Yph Tg(Thy1-YFP)HJrs MGI:6694273
cx6
Ntn4tm1Wjbr/Ntn4tm1Wjbr
Tg(Thy1-YFP)HJrs/0
B6.Cg-Ntn4tm1Wjbr Tg(Thy1-YFP)HJrs MGI:5490632
cx7
Crmp1tm1Pako/Crmp1tm1Pako
Dpysl2tm1.1Gosh/Dpysl2tm1.1Gosh
Tg(Thy1-YFP)HJrs/0
involves: 129 * C57BL/6J * CBA * FVB/N MGI:5312896
cx8
Cx3cr1tm1Litt/Cx3cr1tm1Litt
Psen1tm1Mpm/Psen1tm1Mpm
Tg(APPSwe,tauP301L)1Lfa/0
Tg(Thy1-YFP)HJrs/0
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA MGI:4834196
cx9
Cx3cr1tm1Litt/Cx3cr1+
Psen1tm1Mpm/Psen1tm1Mpm
Tg(APPSwe,tauP301L)1Lfa/0
Tg(Thy1-YFP)HJrs/0
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA MGI:4834197
cx10
Nppctm1.1Fgr/Nppctm1.1Fgr
Tg(Thy1-YFP)HJrs/0
involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * CBA MGI:4365536
cx11
Fat3tm1.2Good/Fat3tm1.2Good
Tg(Thy1-YFP)HJrs/0
involves: 129P2/OlaHsd * C57BL/6J * CBA MGI:5295427
cx12
Dpysl2tm1.1Gosh/Dpysl2tm1.1Gosh
Tg(Thy1-YFP)HJrs/0
involves: 129P2/OlaHsd * C57BL/6J * CBA * FVB/N MGI:5312898
cx13
Kidins220tm1.1Mvc/Kidins220+
Tg(Thy1-YFP)HJrs/0
involves: 129S1/Sv * C57BL/6 * CBA MGI:4820799
cx14
Fjx1tm1Awp/Fjx1+
Tg(Thy1-YFP)HJrs/0
involves: 129X1/SvJ * C57BL/6 * CBA MGI:3771961
cx15
Fjx1tm1Awp/Fjx1tm1Awp
Tg(Thy1-YFP)HJrs/0
involves: 129X1/SvJ * C57BL/6 * CBA MGI:3771959
cx16
Cntn6tm1Kwat/Cntn6tm1Kwat
Tg(Thy1-YFP)HJrs/0
involves: 129X1/SvJ * C57BL/6 * CBA MGI:3820309
cx17
Mbpshi/Mbpshi
Tg(Thy1-YFP)HJrs/0
involves: C3HeB/Fe * C57BL/6 * CBA * SWV MGI:4834581
cx18
St8sia2tm1Jxm/St8sia2tm1Jxm
St8sia4tm1.1Mifu/St8sia4tm1.1Mifu
Tg(Thy1-YFP)HJrs/?
involves: C57BL/6 * CBA MGI:3762775
cx19
Dnajc30tm1(KOMP)Vlcg/Dnajc30tm1(KOMP)Vlcg
Tg(Thy1-YFP)HJrs/0
involves: C57BL/6J * C57BL/6N MGI:6281683


Genotype
MGI:6502664
cn1
Allelic
Composition
Cmtr1tm1b(EUCOMM)Hmgu/Cmtr1tm1b(EUCOMM)Hmgu
Emx1tm1(cre)Krj/Emx1+
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129S2/SvPas * C57BL/6J * C57BL/6N * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cmtr1tm1b(EUCOMM)Hmgu mutation (0 available); any Cmtr1 mutation (51 available)
Emx1tm1(cre)Krj mutation (2 available); any Emx1 mutation (34 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• reduced number of dendritic intersections at a given distance from the soma of cortical layer 5 neuro

immune system
• lack of inflammatory response in Japanese-encephalitis-infected neocortex




Genotype
MGI:3717378
cn2
Allelic
Composition
Ntrk2tm2Lfr/Ntrk2tm2Lfr
Tg(Six3-cre)69Frty/?
Tg(Thy1-YFP)HJrs/?
Genetic
Background
involves: C57BL/6 * CBA * DBA/2
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ntrk2tm2Lfr mutation (1 available); any Ntrk2 mutation (66 available)
Tg(Six3-cre)69Frty mutation (2 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
vision/eye
• at P28, mice have a greater percent of ON-OFF retinal ganglion cells (RGCs) than in control mice
• at P28, mice have fewer ON and OFF RGCs than controls
• at P28, mice have fewer numbers of ON RGC dendritic branches
• at P50, 55.4+/-4.3% of RGCs are bilaminated compared to 37.8+/-1.1% in controls

nervous system
• at P28, mice have fewer numbers of ON retinal ganglion cells (RGCs) dendritic branches
• at P28, mice have a greater percent of ON-OFF retinal ganglion cells (RGCs) than in control mice
• at P28, mice have fewer ON and OFF RGCs than controls
• at P28, mice have fewer numbers of ON RGC dendritic branches
• at P50, 55.4+/-4.3% of RGCs are bilaminated compared to 37.8+/-1.1% in controls




Genotype
MGI:6694271
cx3
Allelic
Composition
Cttnbp2em1Yph/Cttnbp2+
Tg(Thy1-YFP)HJrs/0
Genetic
Background
B6.Cg-Cttnbp2em1Yph Tg(Thy1-YFP)HJrs
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cttnbp2em1Yph mutation (0 available); any Cttnbp2 mutation (93 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• dorsal CA1 neurons have smaller width of dendritic spine heads




Genotype
MGI:6694268
cx4
Allelic
Composition
Cttnbp2em1Yph/Cttnbp2em1Yph
Tg(Thy1-YFP)HJrs/0
Genetic
Background
B6.Cg-Cttnbp2em1Yph Tg(Thy1-YFP)HJrs
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cttnbp2em1Yph mutation (0 available); any Cttnbp2 mutation (93 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• dorsal CA1 neurons have smaller dendritic spines
• dorsal CA1 neurons have fewer dendritic spines
• dorsal CA1 neurons have shorter dendritic spines




Genotype
MGI:6694273
cx5
Allelic
Composition
Cttnbp2em2Yph/Cttnbp2+
Tg(Thy1-YFP)HJrs/0
Genetic
Background
B6.Cg-Cttnbp2em2Yph Tg(Thy1-YFP)HJrs
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cttnbp2em2Yph mutation (0 available); any Cttnbp2 mutation (93 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• dorsal CA1 neurons have reduced density and width of dendritic spines
• dorsal CA1 neurons have reduced density of dendritic spines




Genotype
MGI:5490632
cx6
Allelic
Composition
Ntn4tm1Wjbr/Ntn4tm1Wjbr
Tg(Thy1-YFP)HJrs/0
Genetic
Background
B6.Cg-Ntn4tm1Wjbr Tg(Thy1-YFP)HJrs
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ntn4tm1Wjbr mutation (0 available); any Ntn4 mutation (61 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• retinal ganglion cell (RGC) axons sometimes exhibit defasciculation that forms a fork-like structures before reaching the optic nerve
• fascicles on RGC axons are smaller than in control mice
• retinal ganglion cell axons sometimes exhibit defasciculation that forms a fork-like structures before reaching the optic nerve

vision/eye
• retinal ganglion cell axons sometimes exhibit defasciculation that forms a fork-like structures before reaching the optic nerve

cellular
• retinal ganglion cell (RGC) axons sometimes exhibit defasciculation that forms a fork-like structures before reaching the optic nerve
• fascicles on RGC axons are smaller than in control mice




Genotype
MGI:5312896
cx7
Allelic
Composition
Crmp1tm1Pako/Crmp1tm1Pako
Dpysl2tm1.1Gosh/Dpysl2tm1.1Gosh
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129 * C57BL/6J * CBA * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Crmp1tm1Pako mutation (2 available); any Crmp1 mutation (45 available)
Dpysl2tm1.1Gosh mutation (0 available); any Dpysl2 mutation (34 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• the basal dendrites of layer V pyramidal neurons grow to the apical side with curling and collaterals from apical trunks show disorientation and winding
• the basal dendrites of layer V pyramidal neurons grow to the apical side with curling and collaterals from apical trunks show disorientation and winding
• cortical neurons show an increase in the number of primary dendrites
• the highest number of dendrite crossings is found at about 40 um compared to 60-80 um in controls
• the basal dendritic field is further shifted to the apical part of the cell body and to the close part of cell body compared to mutant mice wild-type for Crmp1




Genotype
MGI:4834196
cx8
Allelic
Composition
Cx3cr1tm1Litt/Cx3cr1tm1Litt
Psen1tm1Mpm/Psen1tm1Mpm
Tg(APPSwe,tauP301L)1Lfa/0
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cx3cr1tm1Litt mutation (6 available); any Cx3cr1 mutation (45 available)
Psen1tm1Mpm mutation (4 available); any Psen1 mutation (48 available)
Tg(APPSwe,tauP301L)1Lfa mutation (3 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• mice do not exhibit the neuron loss or increase in microglial density and migration velocity observed in Cx3cr1tm1Litt/Cx3cr1+ Psen1tm1Mpm/Psen1tm1Mpm Tg(APPSwe,tauP301L)1Lfa mice




Genotype
MGI:4834197
cx9
Allelic
Composition
Cx3cr1tm1Litt/Cx3cr1+
Psen1tm1Mpm/Psen1tm1Mpm
Tg(APPSwe,tauP301L)1Lfa/0
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cx3cr1tm1Litt mutation (6 available); any Cx3cr1 mutation (45 available)
Psen1tm1Mpm mutation (4 available); any Psen1 mutation (48 available)
Tg(APPSwe,tauP301L)1Lfa mutation (3 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• microglial density increases over time around lost neurons unlike in Cx3cr1tm1Litt/Cx3cr1tm1Litt Psen1tm1Mpm/Psen1tm1Mpm Tg(APPSwe,tauP301L)1Lfa mice
• microglia migration velocity around lost neurons is 2-fold greater than in Cx3cr1tm1Litt heterozygotes and homozygotes
• of YFP+ layer III neurons at 4 to 6 months

immune system
• microglial density increases over time around lost neurons unlike in Cx3cr1tm1Litt/Cx3cr1tm1Litt Psen1tm1Mpm/Psen1tm1Mpm Tg(APPSwe,tauP301L)1Lfa mice
• microglia migration velocity around lost neurons is 2-fold greater than in Cx3cr1tm1Litt heterozygotes and homozygotes

hematopoietic system
• microglial density increases over time around lost neurons unlike in Cx3cr1tm1Litt/Cx3cr1tm1Litt Psen1tm1Mpm/Psen1tm1Mpm Tg(APPSwe,tauP301L)1Lfa mice
• microglia migration velocity around lost neurons is 2-fold greater than in Cx3cr1tm1Litt heterozygotes and homozygotes




Genotype
MGI:4365536
cx10
Allelic
Composition
Nppctm1.1Fgr/Nppctm1.1Fgr
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nppctm1.1Fgr mutation (0 available); any Nppc mutation (12 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• at mature stages all sensory axon exhibit a bifurcation failure compared to in wild-type mice




Genotype
MGI:5295427
cx11
Allelic
Composition
Fat3tm1.2Good/Fat3tm1.2Good
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6J * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fat3tm1.2Good mutation (0 available); any Fat3 mutation (241 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• at P3 with ectopic neurites extending towards the outer retina

vision/eye
• at P3 with ectopic neurites extending towards the outer retina




Genotype
MGI:5312898
cx12
Allelic
Composition
Dpysl2tm1.1Gosh/Dpysl2tm1.1Gosh
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6J * CBA * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dpysl2tm1.1Gosh mutation (0 available); any Dpysl2 mutation (34 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• cortical neurons show an increase in the number of primary dendrites
• the basal dendritic field is slightly shifted to the apical side of the cell body




Genotype
MGI:4820799
cx13
Allelic
Composition
Kidins220tm1.1Mvc/Kidins220+
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129S1/Sv * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kidins220tm1.1Mvc mutation (0 available); any Kidins220 mutation (79 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• in vivo, dendritic spines of pyramidal neurons are less stable than spines on wild-type cells
• however, spine formation rate is normal




Genotype
MGI:3771961
cx14
Allelic
Composition
Fjx1tm1Awp/Fjx1+
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fjx1tm1Awp mutation (0 available); any Fjx1 mutation (16 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• in culture, dendrites of hippocampal at day 7 are 50% longer compared to wild-type neurons




Genotype
MGI:3771959
cx15
Allelic
Composition
Fjx1tm1Awp/Fjx1tm1Awp
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fjx1tm1Awp mutation (0 available); any Fjx1 mutation (16 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• dendritic trees of pyramidal cells in the CA1 region of the hippocampus are less complex than in wild-type mice
• the length of dendrites formed by granule cells in the dentate gyrus is increased (197+/-7 um compared to 164+/-7 um in wild-type mice)
• the complexity of apical dendrites in CA1 is reduced
• however, the complexity of granule cell dendrites in the dentate gyrus and the length of apical dendrites are normal
• in culture, dendrites of hippocampal neurons are 56% longer at day 3 and 88% longer at day 7 compared to wild-type neurons and the number of dendrites per cell is increased (1.8+/-0.2 compared to 1.2+/-0.1 for wild-type neurons) without affecting axon length, the number of axons per cell or the number of dendrites per cell




Genotype
MGI:3820309
cx16
Allelic
Composition
Cntn6tm1Kwat/Cntn6tm1Kwat
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cntn6tm1Kwat mutation (1 available); any Cntn6 mutation (64 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mutants exhibit misorientation of apical dendrites in layer V pyramidal neurons in the visual cortex, but not in motor, somatosensory, or auditory cortices or in the upper layer of the visual cortex




Genotype
MGI:4834581
cx17
Allelic
Composition
Mbpshi/Mbpshi
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: C3HeB/Fe * C57BL/6 * CBA * SWV
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbpshi mutation (3 available); any Mbp mutation (32 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA), seizures are more frequent and last longer than in similarly treated wild-type mice
• mice treated with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) exhibit increased axonal damage, excitotoxicity, and behavioral deficits (including seizures, hindlimb and tail paresis, impaired coordination) compared with similarly treated wild-type mice
• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury
• dorsal columns lack myelin unlike in wild-type mice
• mice exhibit amyelinated and hypomyelinated axons unlike in wild-type mice
• mice exhibit amyelinated and hypomyelinated axons unlike in wild-type mice

behavior/neurological
• following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA), seizures are more frequent and last longer than in similarly treated wild-type mice
• on a rotarod at baseline and after treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA)
• hindlimb and tail paresis following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA)

homeostasis/metabolism
• mice treated with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) exhibit increased axonal damage, excitotoxicity, and behavioral deficits (including seizures, hindlimb and tail paresis, impaired coordination) compared with similarly treated wild-type mice
• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury

cellular
• mice treated with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) exhibit increased axonal damage, excitotoxicity, and behavioral deficits (including seizures, hindlimb and tail paresis, impaired coordination) compared with similarly treated wild-type mice
• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury




Genotype
MGI:3762775
cx18
Allelic
Composition
St8sia2tm1Jxm/St8sia2tm1Jxm
St8sia4tm1.1Mifu/St8sia4tm1.1Mifu
Tg(Thy1-YFP)HJrs/?
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
St8sia2tm1Jxm mutation (1 available); any St8sia2 mutation (23 available)
St8sia4tm1.1Mifu mutation (0 available); any St8sia4 mutation (28 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• unlike in wild-type mice, the projections of dendrites and axons from pyramidal cells are disorganized
• the number of pyramidal cells is decreased to 25% of that in wild-type mice




Genotype
MGI:6281683
cx19
Allelic
Composition
Dnajc30tm1(KOMP)Vlcg/Dnajc30tm1(KOMP)Vlcg
Tg(Thy1-YFP)HJrs/0
Genetic
Background
involves: C57BL/6J * C57BL/6N
Cell Lines 16795A-A9
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dnajc30tm1(KOMP)Vlcg mutation (1 available); any Dnajc30 mutation (11 available)
Tg(Thy1-YFP)HJrs mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• the ratio of corpus callosum:neocortex (CC:NCX) is disproportionately lower than that in wild-type controls
• the g-ratio (axon perimeter/axon+myelin perimeter) is significantly reduced, indicating decreased callosal axon thickness
• L5 neocortical pyramidal neurons (PNs) exhibit significantly reduced soma size
• EM analysis of transected corpus callosa revealed that the g-ratio (axon perimeter/axon+myelin perimeter) is significantly reduced, indicating smaller axon calibers
• perforated patch recordings on neocortical pyramidal neurons revealed that the resting membrane potential is significantly more negative than that in wild-type neurons, consistent with lower intracellular ATP levels
• following treatment with tolbutamide (a K+ATP channel inhibitor), pyramidal neurons fail to exhibit a more positive membrane potential, unlike in wild-type neurons
• following treatment with sodium azide (NaN3), a complex IV inhibitor that indirectly decreases ATP, pyramidal neurons fail to exhibit a significant decrease in membrane potential, unlike in wild-type neurons

cellular
• EM analysis of L5 neocortical pyramidal neurons revealed an increase in mitochondrial density and a more rounded shape, consistent with increased mitochondrial fission
• however, cumulative mitochondrial coverage, area, and perimeter remain normal
• L5 neocortical pyramidal neurons exhibit a more rounded mitochondrial shape (decreased aspect ratio)
• L5 neocortical pyramidal neurons exhibit an increase in mitochondrial density
• isolated neocortical mitochondria exhibit reduced ATP production in a luciferase-based ATP synthesis assay
• however, mitochondrial membrane potential and reactive oxygen species (ROS) production are normal
• L5 neocortical pyramidal neurons exhibit dysmorphic mitochondria typical of increased mitochondrial fission

homeostasis/metabolism
• cultured primary neocortical neurons show a significant reduction in basal oxygen consumption rate (OCR) relative to wild-type neurons
• following FCCP treatment to maximize mitochondrial respiration, primary neocortical neurons cannot respire at as high of a rate as wild-type neurons
• however, no differences in OCR are observed in response to oligomycin or antimycinA/rotenone treatment (OXPHOS-inhibiting drugs)





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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory