Skip to main content

Table 1 Comparison of Stable C9orf72 stable mouse and zebrafish mutants showing the distinct phenotype and utility of each model

From: Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features

DNA Construct

Species

RNA Foci

Sense RAN DPR

Antisense RAN DPR

ALS phenotype

FTD like phenotype

Stable or Transient

Phenotype amenable to drug screen

Efficacy of riluzole

Reference

BAC HRE

Mouse

Yes

Yes

No

Yes

Yes

Stable

No

Not tested

[14]

BAC HRE

Mouse

Yes

Yes

No

Yes

Yes

Stable

No

Not tested

[20]

BAC HRE

Mouse

Yes

Yes

No

No

No

Stable

No

Not tested

[29]

BAC HRE

Mouse

Yes

Yes

No

No

No

Stable

No

Not tested

[30]

C9 K/O

Mouse

NA

NA

NA

No

No

Stable

No

NA

[1, 13, 17, 35]

C9 K/D

Zebrafish

NA

NA

NA

Axonal growth defect

No

Transient

No

Not tested

[6]

C9 K/O

Zebrafish

NA

NA

NA

No

No

Stable

No

NA

[34]; (Schmid, Hruscha, Haass, unpublished)

C9 HRE

Zebrafish

Yes

No

No

No (Mild cardiac phenotype)

No

Stable

No

Not tested

[28]

C9-ATG GA

Zebrafish

Yes

No

No

No (Severe cardiac phenotype)

No

Stable

No (lethal)

Not tested

[28]

C9 -ATG HRE

Zebrafish

Yes

Yes

Yes

Yes

Yes

Stable

Yes

Yes

Current manuscript

  1. NA Not applicable, K/O Knockout, K/D Knockdown, Grey boxes represents the features that represent similarity to human ALS/FTD or utility in the high throughput screening of novel therapeutics