Table 2.
Protein levels in human dermal fibroblasts from classic MSUD patients normalized by Hsp60 and represented as percentage respect to the healthy individuals
| Protein levels normalized by Hsp60 | |||||
|---|---|---|---|---|---|
| Sample | E1α | E1β | E2 (%) | E3 (%) | MCAD (%) |
| Patient 1 | n.d | n.d | 152 | 110 | 181 |
| Patient 2 | n.d | n.d | 102 | 106 | 89 |
| Patient 3 | n.d | n.d | 136 | 124 | 124 |
| Patient 4 | n.d | n.d | 100 | 110 | n.d |
n.d., not detected, denotes measurements that were below 95% confidence range of background noise, which was calculated to be 23%, 19%, and 9% of protein level in samples from healthy individuals for E1α (BCKDHA), E1β (BCKDHB), and MCAD (ACADM) respectively.