Research Models

ALS Mouse Models

Dozens of rodents have been generated that model various aspects of ALS, like motor impairment or degeneration of motor neurons. No model recapitulates the human disease perfectly. By organizing information related to the phenotypic characterization of selected ALS models, this resource conveys what is known about each one and facilitates comparison between models. The curation of ALS models was supported in part by a grant from the NINDS.

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Sex-specific differences

34 Models Selected
  • Cortical Neuron Loss
  • Lower Motor Neuron Loss
  • Cytoplasmic Inclusions
  • Gliosis
  • NMJ Abnormalities
  • Muscle Atrophy
  • Motor Impairment
  • Body Weight
  • Premature Death

C9-BAC500 (Brown model)

  • Absent
  • Absent
  • Observed
  • Absent
  • Absent
  • No Data
  • Absent
  • Absent
  • Absent

C9-BACexp (Baloh/Lutz model)

  • Absent
  • Absent
  • Observed
  • Absent
  • Absent
  • Absent
  • Absent
  • Absent
  • Absent

C9orf72 Knock-out

  • Absent
  • Absent
  • Absent
  • Absent
  • Absent
  • Absent
  • Observed
  • Absent
  • Absent

C9ORF72(AAV)(G4C2)66

  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • No Data

Endogenous Sod1 D83G

  • Observed
  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • Observed
  • Observed
  • Observed

FUS-R521C

  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

FUSΔ14 (FUSd14)

  • Absent
  • Absent
  • Observed
  • Absent
  • No Data
  • No Data
  • Absent
  • No Data
  • No Data

FusΔNLS

  • No Data
  • Observed
  • Observed
  • Observed
  • Observed
  • No Data
  • Observed
  • Absent
  • Absent

hFUS (+/+) (PrP-hFUS)

  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

hFUS-P525L

  • No Data
  • Observed
  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • No Data
  • Absent

hFUS-R521C

  • No Data
  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • No Data
  • Absent

hPFN1-G118V

  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

hTDP-43ΔNLS

  • Observed
  • Absent
  • Absent
  • Observed
  • No Data
  • Absent
  • Observed
  • No Data
  • Absent

NEFH-tTA x hTDP-43ΔNLS

  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

PFN1-C71G

  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

PrP-hFUS (R495X)

  • Absent
  • Absent
  • Absent
  • No Data
  • Observed
  • No Data
  • Absent
  • No Data
  • Observed

PrP-hFUS (WT)

  • No Data
  • No Data
  • Absent
  • No Data
  • Absent
  • No Data
  • Absent
  • No Data
  • Observed

SOD1 (G37R)

  • Absent
  • Observed
  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

SOD1-G85R (hybrid)

  • No Data
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

SOD1-G93A (hybrid) (G1H)

  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

TARDBP (A315T) (congenic)

  • No Data
  • Absent
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed
  • Observed

TARDBP (A315T) (hybrid)

  • Observed
  • Observed
  • Observed
  • Observed
  • No Data
  • Observed
  • Observed
  • Observed
  • Observed

TDP-43 (A315T)

  • Absent
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • No Data
  • Absent

TDP-43 (A315T) (line 23)

  • Absent
  • Absent
  • Observed
  • Observed
  • No Data
  • Observed
  • Observed
  • Observed
  • Observed

TDP-43 (G348C)

  • Absent
  • Absent
  • Observed
  • Observed
  • Observed
  • No Data
  • Observed
  • No Data
  • Absent

TDP-43 (M337V)

  • Absent
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed

TDP-43 (M337V) (Mt-TAR6/6)

  • Observed
  • Observed
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed

TDP-43 (Prp)

  • Absent
  • Absent
  • Observed
  • Observed
  • No Data
  • Absent
  • Observed
  • Observed
  • Observed

TDP-43 (Q331K)

  • No Data
  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • Observed
  • No Data
  • No Data

TDP-43 (WT) (Elliott model)

  • Absent
  • Absent
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed
  • Observed

TDP-43 (WT) (Julien model)

  • Absent
  • Absent
  • Absent
  • Observed
  • Observed
  • No Data
  • Observed
  • No Data
  • No Data

TDP-43 (WT) (WT-TAR4/4)

  • Observed
  • Observed
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed

ΔNLS-FUS

  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed

ΔNLS-FUS x TDP-43(WT)

  • Observed
  • Absent
  • Observed
  • Observed
  • No Data
  • No Data
  • Observed
  • Observed
  • Observed
  • Sex-specific differences
  • Cortical Neuron Loss
  • Lower Motor Neuron Loss
  • Cytoplasmic Inclusions
  • Gliosis
  • NMJ Abnormalities
  • Muscle Atrophy
  • Motor Impairment
  • Body Weight
  • Premature Death

C9-BAC500 (Brown model)

Absent

Absent

Observed

Absent

Absent

No Data

Absent

Absent

Absent

C9-BACexp (Baloh/Lutz model)

Absent

Absent

Observed

Absent

Absent

Absent

Absent

Absent

Absent

C9orf72 Knock-out

Absent

Absent

Absent

Absent

Absent

Absent

Observed

Absent

Absent

C9ORF72(AAV)(G4C2)66

Observed

Absent

Observed

Observed

No Data

No Data

Observed

Observed

No Data

Endogenous Sod1 D83G

Observed

Observed

Absent

Observed

Observed

No Data

Observed

Observed

Observed

FUS-R521C

Absent

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

FUSΔ14 (FUSd14)

Absent

Absent

Observed

Absent

No Data

No Data

Absent

No Data

No Data

FusΔNLS

No Data

Observed

Observed

Observed

Observed

No Data

Observed

Absent

Absent

hFUS (+/+) (PrP-hFUS)

Absent

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

hFUS-P525L

No Data

Observed

Absent

Observed

Observed

Observed

Observed

No Data

Absent

hFUS-R521C

No Data

Observed

Absent

Observed

Observed

No Data

No Data

No Data

Absent

hPFN1-G118V

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

hTDP-43ΔNLS

Observed

Absent

Absent

Observed

No Data

Absent

Observed

No Data

Absent

NEFH-tTA x hTDP-43ΔNLS

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

PFN1-C71G

Absent

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

PrP-hFUS (R495X)

Absent

Absent

Absent

No Data

Observed

No Data

Absent

No Data

Observed

PrP-hFUS (WT)

No Data

No Data

Absent

No Data

Absent

No Data

Absent

No Data

Observed

SOD1 (G37R)

Absent

Observed

Absent

Observed

Observed

Observed

Observed

Observed

Observed

SOD1-G85R (hybrid)

No Data

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

SOD1-G93A (hybrid) (G1H)

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

Observed

TARDBP (A315T) (congenic)

No Data

Absent

Observed

Observed

Observed

Observed

Observed

Observed

Observed

TARDBP (A315T) (hybrid)

Observed

Observed

Observed

Observed

No Data

Observed

Observed

Observed

Observed

TDP-43 (A315T)

Absent

Absent

Observed

Observed

No Data

No Data

Observed

No Data

Absent

TDP-43 (A315T) (line 23)

Absent

Absent

Observed

Observed

No Data

Observed

Observed

Observed

Observed

TDP-43 (G348C)

Absent

Absent

Observed

Observed

Observed

No Data

Observed

No Data

Absent

TDP-43 (M337V)

Absent

Absent

Observed

Observed

No Data

No Data

Observed

Observed

Observed

TDP-43 (M337V) (Mt-TAR6/6)

Observed

Observed

Observed

Observed

No Data

No Data

Observed

Observed

Observed

TDP-43 (Prp)

Absent

Absent

Observed

Observed

No Data

Absent

Observed

Observed

Observed

TDP-43 (Q331K)

No Data

Observed

Absent

Observed

Observed

No Data

Observed

No Data

No Data

TDP-43 (WT) (Elliott model)

Absent

Absent

Observed

No Data

No Data

Observed

Observed

Observed

Observed

TDP-43 (WT) (Julien model)

Absent

Absent

Absent

Observed

Observed

No Data

Observed

No Data

No Data

TDP-43 (WT) (WT-TAR4/4)

Observed

Observed

Observed

Observed

No Data

No Data

Observed

Observed

Observed

ΔNLS-FUS

Observed

Absent

Observed

Observed

No Data

No Data

Observed

Observed

Observed

ΔNLS-FUS x TDP-43(WT)

Observed

Absent

Observed

Observed

No Data

No Data

Observed

Observed

Observed