Table 2 Cln3Q352X mice show lowered Cln3 transcript abundance and variable PPT1 and TPP1 activity in multiple tissues. Cln3 transcript abundance of Cln3Q352X mice was significantly lower in all tissues at 1-month of age.

From: A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease

 

Tissue

Male

Female

Ratio to WT (WT = 1)

SEM

p value

Ratio to WT (WT = 1)

SEM

p value

Cln3 transcript abundance

Cerebral cortex

0.6629

0.0696

**0.0062

0.4861

0.03157

**** < 0.0001

Thalamus

0.5452

0.07074

**0.0017

0.3706

0.02216

*0.0316

Cerebellum

0.8023

0.3351

0.6064

0.8707

0.3023

0.6901

Brainstem

0.5549

0.166

*0.0465

0.3361

0.04289

**** < 0.0001

Eye

0.9475

0.04826

0.6637

0.6683

0.05344

*0.0322

Heart

0.9819

0.2918

0.954

0.3409

0.05461

***0.0002

Lung

0.4116

0.1583

*0.0127

0.4777

0.1165

**0.0052

Liver

0.4301

0.05969

***0.0002

0.1991

0.06627

0.0886

Kidney

0.5038

0.04997

***0.0002

0.3799

0.1062

**0.0013

Spleen

0.3524

0.0772

**0.0093

0.5022

0.1768

0.102

Skeletal muscle

0.7366

0.1624

0.1693

0.06647

0.0123

0.3251

PPT1 enzyme activity

Cerebral cortex

0.7661

0.1278

0.1667

0.8603

0.1283

0.4239

Thalamus

1.094

0.03032

0.1569

0.8216

0.2833

0.6876

Cerebellum

1.019

0.06252

0.7108

1.169

0.2876

0.7012

Liver

0.4493

0.04273

***0.0010

0.3517

0.044

**0.0041

Kidney

0.8651

0.08678

0.7256

1.011

0.1025

0.1079

TPP1 enzyme activity

Cerebral cortex

1.433

0.1849

0.0882

1.306

0.1494

0.1818

Thalamus

2.302

0.1929

*0.0108

2.649

0.8586

0.1125

Cerebellum

2.141

0.03622

**** < 0.0001

4.963

1.348

*0.0339

Liver

0.9617

0.08109

0.6927

0.7713

0.05264

0.0693

Kidney

1

0.05606

0.9894

1.119

0.1053

0.3733

  1. Fluorogenic PPT1 and TPP1 enzyme activity assays performed on tissue from 1 month old Cln3Q352X and WT mice present variable PPT1 levels in several tissues of Cln3Q352X mice, with a significant decrease in the liver. An increase in TPP1 enzyme activity was significantly observed in the thalamus and cerebellum in Cln3Q352X mice. Unpaired t test, mean ± SEM, N = 2–4/sex/genotype.
  2. *p < 0.05; **p < 0.01; ***p < 0.001; ****p < 0.0001.
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