Pah-KO

Fig.1 Detection of Pah expression in liver and kidney of 16-week-old male WT and Pah-KO mice by RT-PCR. 

Wild type: one band at 193 bp with primers F1/R1(m Pah) and one band at 178 bp with primers F/R(mGapdh).

Homozygous: no band at 193 bp with primers F1/R1(m Pah) and one band at 178 bp with primers F/R(mGapdh).

Abbr. M, DNA marker; HO, homozygous; WT, wild type.

Fig.2 Detection of Pah mRNA expression in liver and kidney of 16-week-old male WT and Pah-KO mice by crop. 

Abbr. M, DNA marker; HO, homozygous; WT, wild type.

Fig.3 Detection of mouse PAH expression of 16-week-old male WT and Pah-KO mice by WB. 

Abbr. M, marker; WT, wild type; HO, Homozygous.

Fig.4 Characterization of WT C57BL/6 and Pah-KO mice. 

(A) The Pah-KO mice showed lower body weights than the WT mice (n=20/group). (B) Summary of blood Phe at various timepoints is shown (n=20/group). (C) Summary of blood Tyr at various timepoints is shown (n=20/group). Note: Data from the third party.

Fig.5 Hair coat color in WT C57BL/6 and Pah-KO mice. 

Hair coat of the Pah-KO mouse is light brown whereas it is dark brown for the WT C57BL/6 mouse.

Note: Data from the third party.

Fig.6 Brain weight analysis of 16-17-week-old WT and HO Pah-KO mice. (n=3,  Mean ± SEM. t-test, **p < 0.01).

Brain weight analysis showed that the brain weight of Pah-KO mice is significantly reduced. 

Fig.7 LC-MS profiling of serum amino-acid metabolites in Pah-KO mice. 

Relative to WT controls, HO Pah-KO mice exhibited significant changes in L-Phenylalanine (A), L-Tyrosine (B), N-Acetyl-L-Tyrosine (C), Kynurenic acid (D), Succinic acid (E), Argininosuccinic acid (F), 3-Hydroxyhippuric acid (G) and 5-hydroxy-Tryptamine (H) (n = 3, mean ± SEM; Welch’s t-test for A, unpaired t-test for B, D, E and H; *P < 0.05, **P < 0.01, ***P < 0.001).

Abbr. WT, wild type; HO, Homozygous; N/A, Not Available.

Fig.8 Detection of the locomotor ability by pole test. 

Pole test assessment of motor coordination showed that Pah-KO mice took significantly longer to turn (A) and to descend (B) than WT, indicating compromised locomotor performance in the Pah-KO mice. (male, n=8, 8-9 weeks old, Mean ± SEM. Welch's t test for A, Unpaired t test for B, *P < 0.05, **P < 0.01).

Abbr. WT, wild type; HO, Homozygous.

Fig.9 Detection of the cognitive behavior by novel object recognition test. (male, n=8, 8-9 weeks old, Mean ± SEM. Welch's t test).

Abbr. WT, wild type; HO, Homozygous.

Fig.10 Evaluation of the locomotor performance by open field test. 

Representative traces (A), quantification of total distance (B), average speed (C) and immobility time (D) were recorded. Pah-KO mice travelled a shorter distance and moved more slowly than WT mice, whereas immobility remained unchanged. (male, n=8, 8-9 weeks old, Mean ± SEM. Unpaired t test for B, C, D, *p < 0.05).

Abbr. WT, wild type; HO, Homozygous.

Fig.11 Detection of the anxiety-like behavior by open field test. 

Time spent in the corner (A), total distance in the corner (B), time spent in the center (C), total distance in the center (D), number of rearings (E) and duration of rearings were recorded. Pah-KO mice travelled a shorter distance in the center and reared less than WT mice. (male, n=8, 8-9 weeks old, Mean ± SEM. Unpaired t test for A, E and F; Welch's t test for B; Mann-Whitney test for C and D. *p < 0.05, **p < 0.01).

Abbr. WT, wild type; HO, Homozygous.