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Although these patients have extremely low NSP activity, they do not exhibit obvious immunodeficiency symptoms, providing important theoretical support for the safety of DPP1 inhibition therapeutic strategies ( Research indicates that neutrophil function in PLS patients shows specific abnormalities, including: reduced chemotaxis, affecting neutrophil directed migration

Both windows correspond to elevated insulin sensitivity and low circulating insulin, maximizing IGF-1 receptor availability

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The membranes were blocked with 50% Odyssey Blocking Buffer (LI-COR, Lincoln, NE, USA) in TBS containing 0.1% Tween-20, and incubated overnight with primary antibodies

Sensor protein was diluted to a concentration of 0.25 mg mL 1 in TBS buffer to carry out the further analysis