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Figure 7 MRI (T2W) in a 14 days- old baby with cl...

Fig. 2 Diffusion-weighted axial images (superio...


Figure 1 Neuropathology in iMSUD mice detected by...

Figure 10 Proposed model of brain injury and treat...

Figure 6 Low branched-chain amino acid diet prote...

Figure 5 Plasma, brain and liver amino acid chang...

Figure 2 Neuropathology in iMSUD mice by light an...

Figure 9 Norleucine treatment improved survival a...

Figure 8 Norleucine improved survival, behaviour ...

Figure 7 Behavioural and biochemical changes in i...

Figure 4 Neuropathology and limited survival asso...

Figure 3 Limited survival, body and brain weights...

Figure 2 Biochemical characterization of the clas...

Figure 4 Characterization of the intermediate MSU...

Figure 1 E2 gene knockout mouse production. A, Ge...

Figure 3 Transgenic mouse production and characte...

Fig. 1. que mutants exhibit abnormal swimming be...

Fig. 2. The spinal locomotor output is altered i...

Fig. 3. Theque gene encodes dihydrolipoamide bra...

Fig. 4. dbt becomes enriched in the brain and or...

Fig. 5. The free amino acid profile of que mutan...

Fig. 6. que mutants contain a reduced concentrat...

Fig. 7. A working model of how mutation of dbt r...

Figure 1 Flexion of right hip and knee joints, pr...

Figure 2 Extension of both right hip and knee joi...

Fig. 2 Loss of fPP2Cm expression leads to heart...

Fig. 3 Expression and function of PP2Cm in the ...

Fig. 1 Expression pattern of PP2C in mitochondr...

Fig. 4 Potential role played by catabolism of b...

Fig. 10 Axial MR images obtained in a child with...

Fig. 11 A 30-year-old female patient affected by...

Fig. 12 A 32-year-old male patient admitted with...

Fig. 1 A 23-year-old male patient affected by g...

Fig. 3 An 8-month-old child affected by Menkes ...

Fig. 4 Axial diffusion- (a) and T2-weighted ima...

Fig. 5 Magnetic resonance images obtained in an...

Fig. 6 A 53-year-old male patient affected by d...

Fig. 7 Magnetic resonance images obtained durin...
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